Bio

Clinical Focus


  • Pediatric Cardiology

Academic Appointments


Professional Education


  • Board Certification: Adult Congenital Heart Disease, American Board of Internal Medicine (2015)
  • Board Certification: Pediatric Cardiology, American Board of Pediatrics (2006)
  • Fellowship:Boston Children's Hospital (2006) MA
  • Fellowship:Boston Children's Hospital (2005) MA
  • Residency:Children's Hospital of Philadelphia (2002) PA
  • Medical Education:Univ of California San Francisco (1998) CA
  • Board Certification, Adult Congenital Heart Disease, American Board of Internal Medicine (2015)

Teaching

Stanford Advisees


Publications

All Publications


  • Deletion of 22q11 chromosome is associated with postoperative morbidity after unifocalisation surgery. Cardiology in the young Koth, A., Sidell, D., Bauser-Heaton, H., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B., Asija, R. 2018: 1–4

    Abstract

    BACKGROUND: A 22q11 chromosome deletion is common in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals. We sought to determine whether 22q11 chromosome deletion is associated with increased postoperative morbidity after unifocalisation surgery.METHODS: We included all patients with this diagnosis undergoing primary or revision unifocalisation ± ventricular septal defect closure at our institution from 2008 to 2016, and we excluded patients with unknown 22q11 status. Demographic and surgical data were collected. We compared outcomes between those with 22q11 chromosome deletion and those without using non-parametric analysis.RESULTS: We included 180 patients, 41% of whom were documented to have a chromosome 22q11 deletion. Complete unifocalisation was performed in all patients, and intracardiac repair was performed with similar frequency regardless of 22q11 chromosome status. Duration of mechanical ventilation was longer in 22q11 deletion patients. This difference remained significant after adjustment for delayed sternal closure and/or intracardiac repair. Duration of ICU stay was longer in patients with 22q11 deletion, although no longer significant when adjusted for delayed sternal closure and intracardiac repair. Finally, length of hospital stay was longer in 22q11-deleted patients, but this difference was not significant on unadjusted or adjusted analysis.CONCLUSION: Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals and 22q11 deletion are at risk for greater prolonged mechanical ventilation after unifocalisation surgery. Careful attention should be given to the co-morbidities of this population in the perioperative period to mitigate risks that may complicate the postoperative course.

    View details for DOI 10.1017/S1047951118001427

    View details for PubMedID 30160647

  • Safety of ferumoxytol in children undergoing cardiac MRI under general anaesthesia. Cardiology in the young Wise-Faberowski, L., Velasquez, N., Chan, F., Vasanawala, S., McElhinney, D. B., Ramamoorthy, C. 2018; 28 (7): 916–21

    Abstract

    BACKGROUND: Ferumoxytol, an "off-label" contrast agent, allows for better cardiac MRI quality as compared with gadolinium-based contrast agents. However, hypotension has been reported with the use of ferumoxytol for indications other than cardiac MRI. The purpose of our investigation was to evaluate the safety of ferumoxytol in children undergoing general anaesthesia for cardiac MRI.METHODS: Medical records of children undergoing general anaesthesia for cardiac MRI were reviewed. Baseline demographic and medical characteristics, as well as imaging and anaesthetic duration and technique, were collected. The incidence of hypotension or other adverse events', need for vasoactive support, or airway intervention throughout the anaesthetic, was recorded.RESULTS: A total of 95 patients were identified, 61 received ferumoxytol and 34 received gadolinium. There were no significant differences between groups with respect to age, weight, or baseline blood pressure. The incidence of low blood pressure - systolic or mean - after contrast administration did not differ between groups, and there was no difference in sustained hypotension or use of vasopressors between groups. One patient who received ferumoxytol had possible anaphylaxis. The image acquisition time (45 versus 68 min, p=0.002) and anaesthesia duration (100 versus 132 min, p=0.02) were shorter in the ferumoxytol group.CONCLUSION: Transient low blood pressure was common in children undergoing cardiac MRI with anaesthesia, but the incidence of hypotension did not differ between ferumoxytol and gadolinium groups. The use of ferumoxytol was associated with significantly shorter scan time and anaesthesia duration, as well as a decreased need for airway intervention.

    View details for DOI 10.1017/S1047951118000306

    View details for PubMedID 29848399

  • Circulation: Cardiovascular Interventions: The First 10 Years CIRCULATION-CARDIOVASCULAR INTERVENTIONS Faxon, D. P., Leopold, J. A., Abbott, J., McElhinney, D. B., Williams, D. O. 2018; 11 (6): e006901
  • Pathological antibody-mediated rejection in pediatric heart transplant recipients: Immunologic risk factors, hemodynamic significance, and outcomes. Pediatric transplantation Hollander, S. A., Peng, D. M., Mills, M., Berry, G. J., Fedrigo, M., McElhinney, D. B., Almond, C. S., Rosenthal, D. N. 2018: e13197

    Abstract

    Biopsy-diagnosed pAMR has been observed in over half of pediatric HT recipients within 6years of transplantation. We report the incidence and outcomes of pAMR at our center. All endomyocardial biopsies for all HT recipients transplanted between 2010 and 2015 were reviewed and classified using contemporary ISHLT guidelines. Graft dysfunction was defined as a qualitative decrement in systolic function by echocardiogram or an increase of ≥3mmHg in atrial filling pressure by direct measurement. Among 96 patients, pAMR2 occurred in 7 (7%) over a median follow-up period of 3.1years, while no cases of pAMR3 occurred. A history of CHD, DSA at transplant, and elevated filling pressures were associated with pAMR2. Five-sixths (83%) of patients developed new C1q+ DSA at the time of pAMR diagnosis. There was a trend toward reduced survival, with 43% of patients dying within 2.3years of pAMR diagnosis.

    View details for DOI 10.1111/petr.13197

    View details for PubMedID 29729067

  • Association of dead space ventilation and prolonged ventilation after repair of tetralogy of Fallot with pulmonary atresia. The Journal of thoracic and cardiovascular surgery Koth, A. M., Kwiatkowski, D. M., Lim, T. R., Bauser-Heaton, H., Asija, R., McElhinney, D. B., Hanley, F. L., Krawczeski, C. D. 2018

    Abstract

    BACKGROUND: We set out to determine whether patients with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCA) are at risk for elevated dead space ventilation fraction (VD/VT), and whether this is associated with prolonged mechanical ventilation. We hypothesized that elevated VD/VT (>20%) in the first 24hours after unifocalization surgery is associated with increased risk for prolonged mechanical ventilation (>7days).METHODS: All patients with TOF/PA/MAPCA undergoing unifocalization surgery between January 2003 and December 2015 were included in this study. Average VD/VT was calculated over the first 24hours after surgery. Demographic and surgical data were collected. Outcome data included duration of mechanical ventilation. Patients were separated into 2 groups: elevated VD/VT and normal DVSF. Groups were compared using the Student t test, Wilcoxon rank-sum test, and chi2 test. Univariable and multivariable regression analyses were performed with VD/VT as a continuous variable to test for association.RESULTS: Of the 265 included patients, 127 (48%) had an elevated VD/VT. The 2 groups did not differ significantly in any demographic characteristic. Patients with an elevated VD/VT had longer cardiopulmonary bypass times (P=.03), were more likely to have delayed sternal closure, and more likely to have prolonged respiratory failure (odds ratio, 2.2; 95% confidence interval, 1.2-4.0; P=.007). The percent VD/VT was associated with duration of mechanical ventilation in univariable (P<.001) and multivariable (P<.001) regression analyses when controlled for age, weight and bypass time.CONCLUSIONS: Elevated postoperative VD/VT is associated with prolonged mechanical ventilation in patients with TOF/PA/MAPCA following unifocalization. Elevated postoperative VD/VT may be an early indicator of patients who will require prolonged duration of mechanical ventilation, allowing optimization of medical management to promote better outcomes.

    View details for DOI 10.1016/j.jtcvs.2018.04.088

    View details for PubMedID 29884495

  • Pulmonary reinterventions after complete unifocalization and repair in infants and young children with tetralogy of Fallot with major aortopulmonary collaterals JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Bauser-Heaton, H., Borquez, A., Asija, R., Wise-Faberowski, L., Zhang, Y., Downey, L., Perry, S. B., Koth, A., Peng, L. F., Algaze, C. A., Hanley, F. L., McElhinney, D. B. 2018; 155 (4): 1696–1707

    Abstract

    Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) emphasizes unifocalization and augmentation of the reconstructed pulmonary arterial (PA) circulation and complete intracardiac repair in infancy, usually in a single procedure. This approach yields a high rate of complete repair with excellent survival and low right ventricular (RV) pressure. However, little is known about remodeling of the unifocalized and reconstructed pulmonary circulation or about reinterventions on the reconstructed PAs or the RV outflow tract conduit.We reviewed patients who underwent complete repair of TOF with MAPCAs at our center at <2 years of age, either as a single-stage procedure or after previous procedures. Outcomes included freedom from conduit or PA intervention after repair, which were assessed by Cox regression and Kaplan-Meier analysis.The study cohort included 272 patients. There were 6 early deaths and a median of follow-up of 3.6 years after complete repair. Reinterventions on the pulmonary circulation were performed in 134 patients, including conduit interventions in 101 patients, branch PA interventions in 101, and closure of residual MAPCAs in 9. The first conduit reintervention consisted of surgical conduit replacement in 77 patients, transcatheter pulmonary valve replacement with a Melody valve in 14, and angioplasty or bare metal stenting in 10. Surgical PA reinterventions were performed in 46 patients and transcatheter reinterventions in 75 (both in 20). Most PA reinterventions involved a single lung, and most transcatheter reinterventions a single vessel. Freedom from conduit replacement or transcatheter pulmonary valve replacement was 70 ± 3% at 5 years and was shorter in patients with smaller initial conduit size. Freedom from any PA reintervention was 64 ± 3% at 5 years, with the greatest rate during the first year. On multivariable analysis, factors associated with longer freedom from any PA reintervention included lower postrepair RV:aortic pressure ratio and larger original conduit size.We were able to obtain follow-up data for the majority of patients, which demonstrated freedom from PA reintervention for two thirds of patients. The time course of and risk factors for conduit reintervention in this cohort appeared similar to previously reported findings in patients who received RV-PA conduits in early childhood for other anomalies. Relative to the severity of baseline pulmonary vascular anatomy in TOF with MAPCAs, reinterventions on the reconstructed PAs were uncommon after repair according to our approach, and major reinterventions were rare. Nevertheless, PA reinterventions are an important aspect of the overall management strategy.

    View details for DOI 10.1016/j.jtcvs.2017.11.086

    View details for Web of Science ID 000427633800082

    View details for PubMedID 29352588

  • Interstage evaluation of homograft-valved right ventricle to pulmonary artery conduits for palliation of hypoplastic left heart syndrome JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Sandeep, N., Punn, R., Balasubramanian, S., Smith, S. N., Reinhartz, O., Zhang, Y., Wright, G. E., Peng, L. F., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B. 2018; 155 (4): 1747-+

    Abstract

    Palliation of hypoplastic left heart syndrome with a standard nonvalved right ventricle to pulmonary artery conduit results in an inefficient circulation in part due to diastolic regurgitation. A composite right ventricle pulmonary artery conduit with a homograft valve has a hypothetical advantage of reducing regurgitation, but may differ in the propensity for stenosis because of valve remodeling.This retrospective cohort study included 130 patients with hypoplastic left heart syndrome who underwent a modified stage 1 procedure with a right ventricle to pulmonary artery conduit from 2002 to 2015. A composite valved conduit (cryopreserved homograft valve anastomosed to a polytetrafluoroethylene tube) was placed in 100 patients (47 aortic, 32 pulmonary, 13 femoral/saphenous vein, 8 unknown), and a nonvalved conduit was used in 30 patients. Echocardiographic functional parameters were evaluated before and after stage 1 palliation and before the bidirectional Glenn procedure, and interstage interventions were assessed.On competing risk analysis, survival over time was better in the valved conduit group (P = .040), but this difference was no longer significant after adjustment for surgical era. There was no significant difference between groups in the cumulative incidence of bidirectional Glenn completion (P = .15). Patients with a valved conduit underwent more interventions for conduit obstruction in the interstage period, but this difference did not reach significance (P = .16). There were no differences between groups in echocardiographic parameters of right ventricle function at baseline or pre-Glenn.In this cohort of patients with hypoplastic left heart syndrome, inclusion of a valved right ventricle to pulmonary artery conduit was not associated with any difference in survival on adjusted analysis and did not confer an identifiable benefit on right ventricle function.

    View details for DOI 10.1016/j.jtcvs.2017.11.001

    View details for Web of Science ID 000427633800092

    View details for PubMedID 29223842

  • Transcatheter Pulmonary Valve Replacement With the Melody Valve in Small Diameter Expandable Right Ventricular Outflow Tract Conduits JACC-CARDIOVASCULAR INTERVENTIONS Shahanavaz, S., Qureshi, A. M., Levi, D. S., Boudjemline, Y., Peng, L. F., Martin, M., Bauser-Heaton, H., Keeshan, B., Asnes, J. D., Jones, T. K., Justino, H., Aboulhosn, J. A., Gray, R. G., Nguyen, H., Balzer, D. T., McElhinney, D. B. 2018; 11 (6): 554–64

    Abstract

    This study sought to evaluate the safety, feasibility, and outcomes of transcatheter pulmonary valve replacement (TPVR) in conduits ≤16 mm in diameter.The Melody valve (Medtronic, Minneapolis, Minnesota) is approved for the treatment of dysfunctional right ventricular outflow tract (RVOT) conduits ≥16 mm in diameter at the time of implant. Limited data are available regarding the use of this device in smaller conduits.The study retrospectively evaluated patients from 9 centers who underwent percutaneous TPVR into a conduit that was ≤16 mm in diameter at the time of implant, and reported procedural characteristics and outcomes.A total of 140 patients were included and 117 patients (78%; median age and weight 11 years of age and 35 kg, respectively) underwent successful TPVR. The median original conduit diameter was 15 (range: 9 to 16) mm, and the median narrowest conduit diameter was 11 (range: 4 to 23) mm. Conduits were enlarged to a median diameter of 19 mm (29% larger than the implanted diameter), with no difference between conduits. There was significant hemodynamic improvement post-implant, with a residual peak RVOT pressure gradient of 7 mm Hg (p < 0.001) and no significant pulmonary regurgitation. During a median follow-up of 2.0 years, freedom from RVOT reintervention was 97% and 89% at 2 and 4 years, respectively, and there were no deaths and 5 cases of endocarditis (incidence rate 2.0% per patient-year).In this preliminary experience, TPVR with the Melody valve into expandable small diameter conduits was feasible and safe, with favorable early and long-term procedural and hemodynamic outcomes.

    View details for DOI 10.1016/j.jcin.2018.01.239

    View details for Web of Science ID 000428977200009

    View details for PubMedID 29566801

  • QUALITY OF INR CONTROL IN A CENTRALIZED PHARMACIST-LED PEDIATRIC CARDIOLOGY WARFARIN MANAGEMENT PROGRAM Lee, J., Murray, J., Wilkens, S., Zhang, Y., Nishioka, F., Rosenthal, D., Roth, S., Murphy, D., McElhinney, D., Almond, C. ELSEVIER SCIENCE INC. 2018: 609
  • Anesthetic Management During Surgery for Tetralogy of Fallot With Pulmonary Atresia and Major Aortopulmonary Collateral Arteries WORLD JOURNAL FOR PEDIATRIC AND CONGENITAL HEART SURGERY Quinonez, Z. A., Downey, L., Abbasi, R. K., Kuan, C., Asija, R., McElhinney, D. B., Hanley, F. L., Mainwaring, R. D., Wise-Faberowski, L. 2018; 9 (2): 236–41

    Abstract

    Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals (TOF/PA/MAPCAs) is a heterogeneous disease with varying degrees of severity, requiring complex anesthetic management. Our institution has adopted the approach of early complete repair with incorporation of all lung segments, extensive lobar and branch segmental pulmonary artery reconstruction, and ventricular septal defect closure. While the surgical management of TOF/PA/MAPCAs has been extensively described and varies depending on the institution, there is a paucity of literature on the anesthetic management for such procedures. Herein, we describe our anesthetic management based on our own institution's surgical approach at Lucile Packard Children's Hospital/Stanford University.

    View details for DOI 10.1177/2150135118754522

    View details for Web of Science ID 000429980300017

    View details for PubMedID 29544416

  • Percutaneous transcatheter pulmonary valve replacement in children weighing less than 20 kg CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Martin, M., Shahanavaz, S., Peng, L. F., Asnes, J. D., Riley, M., Hellenbrand, W. E., Balzer, D. T., Gray, R. G., McElhinney, D. B. 2018; 91 (3): 485–94

    Abstract

    Since FDA approval of the Melody valve, transcatheter pulmonary valve replacement (TPVR) has been offered to an expanding population. Limited data exist regarding the safety and feasibility of TPVR in smaller patients.All patients weighing <20 kg who underwent catheterization for percutaneous TPVR at four centers were reviewed.Of the 51 patients reviewed, 43 (84%) had successful valve implantation and 8 (16%) were found not to be candidates due to access veins too small (n = 3), coronary artery/aortic root compression (n = 3), and RVOT too large (n = 2). The 43 patients who underwent successful percutaneous TPVR had a median age and weight of 5.8 years (3.3-10) and 17.7 kg (13.5-19.8), and most had tetralogy of Fallot (TOF). TPVR was performed via femoral vein (FV) access in 24 patients and internal jugular vein (IJV) access in 19 patients. Median weight in patients with IJV implant was 1.1 kg lower than those who underwent transfemoral implant (17.0 vs. 18.1 kg, P = 0.05). There were four adverse events: one iliac vein injury, one contained MPA tear, and two patients with post-procedure femoral vein bleeding. All patients were alive at recent follow-up, a median of 2.0 years (0.1-6.0) after TPVR. There was excellent valve function with mean Doppler gradients of 3-20 mm Hg, and trivial or mild pulmonary regurgitation in all but one patient. There were no RVOT reinterventions and no cases of endocarditis.Percutaneous TPVR can be safely performed in patients <20 kg. The procedure frequently requires IJV access, and access site/bleeding complications may be more common in this cohort.

    View details for DOI 10.1002/ccd.27432

    View details for Web of Science ID 000425509700022

    View details for PubMedID 29193671

  • Assessing Statewide All-Cause Future One-Year Mortality: Prospective Study With Implications for Quality of Life, Resource Utilization, and Medical Futility. Journal of medical Internet research Guo, Y., Zheng, G., Fu, T., Hao, S., Ye, C., Zheng, L., Liu, M., Xia, M., Jin, B., Zhu, C., Wang, O., Wu, Q., Culver, D. S., Alfreds, S. T., Stearns, F., Kanov, L., Bhatia, A., Sylvester, K. G., Widen, E., McElhinney, D. B., Ling, X. B. 2018; 20 (6): e10311

    Abstract

    For many elderly patients, a disproportionate amount of health care resources and expenditures is spent during the last year of life, despite the discomfort and reduced quality of life associated with many aggressive medical approaches. However, few prognostic tools have focused on predicting all-cause 1-year mortality among elderly patients at a statewide level, an issue that has implications for improving quality of life while distributing scarce resources fairly.Using data from a statewide elderly population (aged ≥65 years), we sought to prospectively validate an algorithm to identify patients at risk for dying in the next year for the purpose of minimizing decision uncertainty, improving quality of life, and reducing futile treatment.Analysis was performed using electronic medical records from the Health Information Exchange in the state of Maine, which covered records of nearly 95% of the statewide population. The model was developed from 125,896 patients aged at least 65 years who were discharged from any care facility in the Health Information Exchange network from September 5, 2013, to September 4, 2015. Validation was conducted using 153,199 patients with same inclusion and exclusion criteria from September 5, 2014, to September 4, 2016. Patients were stratified into risk groups. The association between all-cause 1-year mortality and risk factors was screened by chi-squared test and manually reviewed by 2 clinicians. We calculated risk scores for individual patients using a gradient tree-based boost algorithm, which measured the probability of mortality within the next year based on the preceding 1-year clinical profile.The development sample included 125,896 patients (72,572 women, 57.64%; mean 74.2 [SD 7.7] years). The final validation cohort included 153,199 patients (88,177 women, 57.56%; mean 74.3 [SD 7.8] years). The c-statistic for discrimination was 0.96 (95% CI 0.93-0.98) in the development group and 0.91 (95% CI 0.90-0.94) in the validation cohort. The mortality was 0.99% in the low-risk group, 16.75% in the intermediate-risk group, and 72.12% in the high-risk group. A total of 99 independent risk factors (n=99) for mortality were identified (reported as odds ratios; 95% CI). Age was on the top of list (1.41; 1.06-1.48); congestive heart failure (20.90; 15.41-28.08) and different tumor sites were also recognized as driving risk factors, such as cancer of the ovaries (14.42; 2.24-53.04), colon (14.07; 10.08-19.08), and stomach (13.64; 3.26-86.57). Disparities were also found in patients' social determinants like respiratory hazard index (1.24; 0.92-1.40) and unemployment rate (1.18; 0.98-1.24). Among high-risk patients who expired in our dataset, cerebrovascular accident, amputation, and type 1 diabetes were the top 3 diseases in terms of average cost in the last year of life.Our study prospectively validated an accurate 1-year risk prediction model and stratification for the elderly population (≥65 years) at risk of mortality with statewide electronic medical record datasets. It should be a valuable adjunct for helping patients to make better quality-of-life choices and alerting care givers to target high-risk elderly for appropriate care and discussions, thus cutting back on futile treatment.

    View details for DOI 10.2196/10311

    View details for PubMedID 29866643

  • Prediction of Incident Hypertension Within the Next Year: Prospective Study Using Statewide Electronic Health Records and Machine Learning. Journal of medical Internet research Ye, C., Fu, T., Hao, S., Zhang, Y., Wang, O., Jin, B., Xia, M., Liu, M., Zhou, X., Wu, Q., Guo, Y., Zhu, C., Li, Y. M., Culver, D. S., Alfreds, S. T., Stearns, F., Sylvester, K. G., Widen, E., McElhinney, D., Ling, X. 2018; 20 (1): e22

    Abstract

    As a high-prevalence health condition, hypertension is clinically costly, difficult to manage, and often leads to severe and life-threatening diseases such as cardiovascular disease (CVD) and stroke.The aim of this study was to develop and validate prospectively a risk prediction model of incident essential hypertension within the following year.Data from individual patient electronic health records (EHRs) were extracted from the Maine Health Information Exchange network. Retrospective (N=823,627, calendar year 2013) and prospective (N=680,810, calendar year 2014) cohorts were formed. A machine learning algorithm, XGBoost, was adopted in the process of feature selection and model building. It generated an ensemble of classification trees and assigned a final predictive risk score to each individual.The 1-year incident hypertension risk model attained areas under the curve (AUCs) of 0.917 and 0.870 in the retrospective and prospective cohorts, respectively. Risk scores were calculated and stratified into five risk categories, with 4526 out of 381,544 patients (1.19%) in the lowest risk category (score 0-0.05) and 21,050 out of 41,329 patients (50.93%) in the highest risk category (score 0.4-1) receiving a diagnosis of incident hypertension in the following 1 year. Type 2 diabetes, lipid disorders, CVDs, mental illness, clinical utilization indicators, and socioeconomic determinants were recognized as driving or associated features of incident essential hypertension. The very high risk population mainly comprised elderly (age>50 years) individuals with multiple chronic conditions, especially those receiving medications for mental disorders. Disparities were also found in social determinants, including some community-level factors associated with higher risk and others that were protective against hypertension.With statewide EHR datasets, our study prospectively validated an accurate 1-year risk prediction model for incident essential hypertension. Our real-time predictive analytic model has been deployed in the state of Maine, providing implications in interventions for hypertension and related diseases and hopefully enhancing hypertension care.

    View details for DOI 10.2196/jmir.9268

    View details for PubMedID 29382633

  • Bronchoscopy in children with tetralogy of fallot, pulmonary atresia, and major aortopulmonary collaterals. Pediatric pulmonology Sidell, D. R., Koth, A. M., Bauser-Heaton, H., McElhinney, D. B., Wise-Faberowski, L., Tracy, M. C., Hanley, F. L., Asija, R. 2017

    Abstract

    Children with Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals (TOF/PA/MAPCAs) undergoing unifocalization surgery are at risk for developing more postoperative respiratory complications than children undergoing other types of congenital heart surgery. Bronchoscopy is used in the perioperative period for diagnostic and therapeutic purposes. In this study, we describe bronchoscopic findings and identify factors associated with selection for bronchoscopy.Retrospective case-control.All patients with TOF/PA/MAPCAs who underwent unifocalization surgery from September 2005 through March 2016 were included. Patients who underwent bronchoscopy in the perioperative period were compared to a randomly selected cohort of 172 control patients who underwent unifocalization without bronchoscopy during the study period.Forty-three children underwent perioperative bronchoscopy at a median of 9 days postoperatively. Baseline demographics were similar in bronchoscopy patients and controls. Patients who underwent bronchoscopy were more likely to have a chromosome 22q11 deletion and were more likely have undergone unifocalization surgery without intracardiac repair. These patients had a longer duration of mechanical ventilation, ICU duration, and length of hospitalization. Abnormalities were detected on bronchoscopy in 35 patients (81%), and 20 (35%) of bronchoscopy patients underwent a postoperative intervention related to abnormalities identified on bronchoscopy.Bronchoscopy is a useful therapeutic and diagnostic instrument for children undergoing unifocalization surgery, capable of identifying abnormalities leading to an additional intervention in over one third of patients. Special attention should be given to children with a 22q11 deletion to expedite diagnosis and intervention for possible airway complications.

    View details for DOI 10.1002/ppul.23732

    View details for PubMedID 28504356

  • Cerebral Oxygen Saturation in Children With Congenital Heart Disease and Chronic Hypoxemia. Anesthesia and analgesia Kussman, B. D., Laussen, P. C., Benni, P. B., McGowan, F. X., McElhinney, D. B. 2017

    Abstract

    Increased hemoglobin (Hb) concentration accompanying hypoxemia is a compensatory response to maintain tissue oxygen delivery. Near infrared spectroscopy (NIRS) is used clinically to detect abnormalities in the balance of cerebral tissue oxygen delivery and consumption, including in children with congenital heart disease (CHD). Although NIRS-measured cerebral tissue O2 saturation (ScO2) correlates with arterial oxygen saturation (SaO2), jugular bulb O2 saturation (SjbO2), and Hb, little data exist on the interplay between these factors and cerebral O2 extraction (COE). This study investigated the associations of ScO2 and ΔSaO2-ScO2 with SaO2 and Hb and verified the normal range of ScO2 in children with CHD.Children undergoing cardiac catheterization for CHD were enrolled in a calibration and validation study of the FORE-SIGHT NIRS monitor. Two pairs of simultaneous arterial and jugular bulb samples were drawn for co-oximetry, calculation of a reference ScO2 (REF CX), and estimation of COE. Pearson correlation and linear regression were used to determine relationships between O2 saturation parameters and Hb. Data were also analyzed according to diagnostic group defined as acyanotic (SaO2 ≥ 90%) and cyanotic (SaO2 < 90%).Of 65 children studied, acceptable jugular bulb samples (SjbO2 absolute difference between samples ≤10%) were obtained in 57 (88%). The ΔSaO2-SjbO2, ΔSaO2-ScO2, and ΔSaO2-REF CX were positively correlated with SaO2 and negatively correlated with Hb (all P < .001). Although by diagnostic group ScO2 differed statistically (P = .002), values in the cyanotic patients were within the range considered normal (69% ± 6%). COE estimated by the difference between arterial and jugular bulb O2 content (ΔCaO2-CjbO2, mL O2/100 mL) was not different for cyanotic and acyanotic patients (P = .10), but estimates using ΔSaO2-SjbO2, ΔSaO2-ScO2, or ΔSaO2-ScO2/SaO2 were significantly different between the cyanotic and acyanotic children (P < .001).Children with adequately compensated chronic hypoxemia appear to have ScO2 values within the normal range. The ΔSaO2-ScO2 is inversely related to Hb, with the implication that in the presence of reduced Hb, particularly if coupled with a decreased cardiac output, the ScO2 can fall to values associated with brain injury in laboratory studies.

    View details for DOI 10.1213/ANE.0000000000002073

    View details for PubMedID 28514318

  • Transcatheter interventions in adults with congenital heart disease: Surveys from the Society for Cardiovascular Angiography and Interventions to identify current patterns of care and perception on training requirements. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Wadia, S. K., Accavitti, M. J., Morgan, G. J., Kenny, D., Hijazi, Z. M., Jones, T. K., Cabalka, A. K., McElhinney, D. B., Kavinsky, C. J. 2017

    Abstract

    Interventional catheterization is central to the care of Adults with Congenital Heart Disease (ACHD). Current standards for care provision and training in ACHD intervention are lacking. We sought to examine trends in current practice and training among interventionalists.We analyzed the results of two separate international surveys in June 2016. One was sent to all active members from the Society of Cardiovascular Angiography and Interventions (SCAI) who self-identified Structural Heart Disease or Congenital Heart Disease as a practice area. The second survey was conducted through the Pediatric Interventional Early Career Society (PICES) aimed at pediatric and adult congenital interventionalists in their first seven years after training. The total survey sample sizes were 1,535 and 112, respectively.Response rates for the SCAI and PICES surveys were 15% (237/1,535) and 75% (84/112), respectively. Most respondents (74%) worked at institutions with pediatric and adult facilities in proximity (major medical centers). While 75% of SCAI respondents believed complex transcatheter procedures should be performed by ACHD-trained interventionalists or multidisciplinary teams, only 32% reported such care is being provided at the present time. Most pediatric and adult cardiologists surveyed (73%) do not believe current interventional fellowships provide adequate training for proficiency in ACHD interventions.ACHD management remains underdeveloped in relative terms, particularly in the United States. Significant gaps in interventional standards of practice and future training needs were recognized by this study. Our survey should serve as an impetus to establish training guidelines for physicians who seek to perform ACHD interventions.

    View details for DOI 10.1002/ccd.27151

    View details for PubMedID 28493591

  • Tricuspid Regurgitation Does Not Impact Right Ventricular Remodeling After Percutaneous Pulmonary Valve Implantation JACC-CARDIOVASCULAR INTERVENTIONS Tanase, D., Ewert, P., Georgiev, S., Meierhofer, C., von Ohain, J. P., McElhinney, D. B., Hager, A., Kuehn, A., Eicken, A. 2017; 10 (7): 701-708
  • Tricuspid Regurgitation Does Not Impact Right Ventricular Remodeling After Percutaneous Pulmonary Valve Implantation. JACC. Cardiovascular interventions Tanase, D., Ewert, P., Georgiev, S., Meierhofer, C., Pabst von Ohain, J., McElhinney, D. B., Hager, A., Kühn, A., Eicken, A. 2017; 10 (7): 701-708

    Abstract

    This study sought to investigate the impact of tricuspid regurgitation (TR) on right ventricular function after percutaneous pulmonary valve implantation (PPVI).PPVI provides a less invasive alternative to surgery in patients with right ventricular-to-pulmonary artery (RV-PA) conduit dysfunction. Recovery of the right ventricle has been described after PPVI for patients with pulmonary stenosis and for those with pulmonary regurgitation. Additional TR enforces RV dysfunction by supplemental volume overload. Limited data are available on the potential of the right ventricle to recover in such a specific hemodynamic situation.In a matched cohort study, we compared patients who underwent PPVI with additional TR with those without TR.The degree of TR improved in 83% of the patients. In our patients (n = 36) exercise capacity and right ventricular volume index improved similarly 6 months after PPVI in patients with and without important TR. None of them had significant TR in the long-term follow-up of median 78 months.PPVI improves not only RV-PA-conduit dysfunction, but also concomitant TR. In patients with a dysfunctional RV-PA conduit and TR, the decision whether to fix TR should be postponed after PPVI.

    View details for DOI 10.1016/j.jcin.2017.01.036

    View details for PubMedID 28385408

  • Programmatic Approach to Management of Tetralogy of Fallot With Major Aortopulmonary Collateral Arteries A 15-Year Experience With 458 Patients CIRCULATION-CARDIOVASCULAR INTERVENTIONS Bauser-Heaton, H., Borquez, A., Han, B., Ladd, M., Asija, R., Downey, L., Koth, A., Algaze, C. A., Wise-Faberowski, L., Perry, S. B., Shin, A., Peng, L. F., Hanley, F. L., McElhinney, D. B. 2017; 10 (4)

    Abstract

    Tetralogy of Fallot with major aortopulmonary collateral arteries is a complex and heterogeneous condition. Our institutional approach to this lesion emphasizes early complete repair with the incorporation of all lung segments and extensive lobar and segmental pulmonary artery reconstruction.We reviewed all patients who underwent surgical intervention for tetralogy of Fallot and major aortopulmonary collateral arteries at Lucile Packard Children's Hospital Stanford (LPCHS) since November 2001. A total of 458 patients underwent surgery, 291 (64%) of whom underwent their initial procedure at LPCHS. Patients were followed for a median of 2.7 years (mean 4.3 years) after the first LPCHS surgery, with an estimated survival of 85% at 5 years after first surgical intervention. Factors associated with worse survival included first LPCHS surgery type other than complete repair and Alagille syndrome. Of the overall cohort, 402 patients achieved complete unifocalization and repair, either as a single-stage procedure (n=186), after initial palliation at our center (n=74), or after surgery elsewhere followed by repair/revision at LPCHS (n=142). The median right ventricle:aortic pressure ratio after repair was 0.35. Estimated survival after repair was 92.5% at 10 years and was shorter in patients with chromosomal anomalies, older age, a greater number of collaterals unifocalized, and higher postrepair right ventricle pressure.Using an approach that emphasizes early complete unifocalization and repair with incorporation of all pulmonary vascular supply, we have achieved excellent results in patients with both native and previously operated tetralogy of Fallot and major aortopulmonary collateral arteries.

    View details for DOI 10.1161/CIRCINTERVENTIONS.116.004952

    View details for Web of Science ID 000397579800001

    View details for PubMedID 28356265

  • Development and validation of a major adverse transplant event (MATE) score to predict late graft loss in pediatric heart transplantation. journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Almond, C. S., Hoen, H., Rossano, J. W., Castleberry, C., Auerbach, S. R., Yang, L., Lal, A. K., Everitt, M. D., Fenton, M., Hollander, S. A., Pahl, E., Pruitt, E., Rosenthal, D. N., McElhinney, D. B., Daly, K. P., Desai, M. 2017

    Abstract

    There is inadequate power to perform a valid clinical trial in pediatric heart transplantation (HT) using a conventional end-point, because the disease is rare and hard end-points, such as death or graft loss, are infrequent. We sought to develop and validate a surrogate end-point involving the cumulative burden of post-transplant complications to predict death/graft loss to power a randomized clinical trial of maintenance immunosuppression in pediatric HT.Pediatric Heart Transplant Study (PHTS) data were used to identify all children who underwent an isolated orthotopic HT between 2005 and 2014 who survived to 6 months post-HT. A time-varying Cox model was used to develop and evaluate a surrogate end-point comprised of 6 major adverse transplant events (MATEs) (acute cellular rejection [ACR], antibody-mediated rejection [AMR], infection, cardiac allograft vasculopathy [CAV], post-transplant lymphoproliferative disease [PTLD] and chronic kidney disease [CKD]) occurring between 6 and 36 months, where individual events were defined according to international guidelines. Two thirds of the study cohort was used for score development, and one third of the cohort was used to test the score.Among 2,118 children, 6.4% underwent graft loss between 6 and 36 months post-HT, whereas 39% developed CKD, 34% ACR, 34% infection, 9% AMR, 4% CAV and 2% PTLD. The best predictive score involved a simple MATE score sum, yielding a concordance probability estimate (CPE) statistic of 0.74. Whereas the power to detect non-inferiority (NI), assuming the NI hazard ratio of 1.45 in graft survival was 10% (assuming 200 subjects and 6% graft loss rate), the power to detect NI assuming a 2-point non-inferiority margin was >85% using the MATE score.The MATE score reflects the cumulative burden of MATEs and has acceptable prediction characteristics for death/graft loss post-HT. The MATE score may be useful as a surrogate end-point to power a clinical trial in pediatric HT.

    View details for DOI 10.1016/j.healun.2017.03.013

    View details for PubMedID 28465118

  • A multicenter study of the impella device for mechanical support of the systemic circulation in pediatric and adolescent patients. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Dimas, V. V., Morray, B. H., Kim, D. W., Almond, C. S., Shahanavaz, S., Tume, S. C., Peng, L. F., McElhinney, D. B., Justino, H. 2017

    Abstract

    The objective was to review the use of Impella devices (Abiomed Inc, Danvers, MA) for temporary circulatory support in pediatric and adolescent patients (age ≤ 21 yrs).Options for minimally invasive circulatory support in children are limited, and published data are confined to case reports and small case series.This was a retrospective, multicenter review of Impella implants in pediatric and adolescent patients from 2009-15, using standardized data collection and INTERMACS definitions.A total of 39 implants were performed in 38 patients from 16 centers. Median age and weight were 16 yrs (4-21 yrs) and 62 kg (15-134 kg). The primary indication for implant was cardiogenic shock in 28 patients (72%). Cardiac allograft rejection, myocarditis, or cardiomyopathy were the underlying diagnosis in 23 patients (59%); 11 patients had congenital heart disease. The median duration of support was 45 hr (1-1224 hr). Indications for explant included ventricular recovery in 16 patients, transition to another device in 12, death in 5, and transplant in 1. Survival was 85% at 7 days and 68% at 30 days. Major adverse events occurred in 8 patients: hemolysis in 3, bleeding in 2, stroke in 1 (unclear if related to Impella), sepsis in 1, and critical leg ischemia in 1. An increase in aortic regurgitation was noted in three patients, with no evidence of valve injury.Temporary circulatory support with Impella devices is feasible in pediatric and adolescent patients, with acceptable risk profiles. More experience and follow up is needed to improve technical performance and patient selection. © 2017 Wiley Periodicals, Inc.

    View details for DOI 10.1002/ccd.26973

    View details for PubMedID 28295963

  • Functional status of United States children supported with a left ventricular assist device at heart transplantation. journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Bulic, A., Maeda, K., Zhang, Y., Chen, S., McElhinney, D. B., Dykes, J. C., Hollander, A. M., Hollander, S. A., Murray, J., Reinhartz, O., Gowan, M. A., Rosenthal, D. N., Almond, C. S. 2017

    Abstract

    As survival with pediatric left ventricular assist devices (LVADs) has improved, decisions regarding the optimal support strategy may depend more on quality of life and functional status (FS) rather than mortality alone. Limited data are available regarding the FS of children supported with LVADs. We sought to compare the FS of children supported with LVADs vs vasoactive infusions to inform decision making around support strategies.Organ Procurement and Transplant Network data were used to identify all United States children aged between 1 and 21 years at heart transplant (HT) between 2006 and 2015 for dilated cardiomyopathy and supported with an LVAD or vasoactive infusions alone at HT. FS was measured using the 10-point Karnofsky and Lansky scale.Of 701 children who met the inclusion criteria, 430 (61%) were supported with vasoactive infusions, and 271 (39%) were supported with an LVAD at HT. Children in the LVAD group had higher median FS scores at HT than children in the vasoactive infusion group (6 vs 5, p < 0.001) but lower FS scores at listing (4 vs 6, p < 0.001). The effect persisted regardless of patient location at HT (home, hospital, intensive care) or device type. Discharge by HT occurred in 46% of children in the LVAD group compared with 26% of children in the vasoactive infusion cohort (p = 0.001). Stroke was reported at HT in 3% of children in the LVAD cohort and in 1% in the vasoactive infusion cohort (p = 0.04).Among children with dilated cardiomyopathy undergoing HT, children supported with LVADs at HT have higher FS than children supported with vasoactive infusions at HT, regardless of device type or hospitalization status. Children supported with LVADs at HT were more likely to be discharged from the hospital but had a higher prevalence of stroke at HT.

    View details for DOI 10.1016/j.healun.2017.02.024

    View details for PubMedID 28363739

  • Rehospitalization after pediatric heart transplantation: Incidence, indications, and outcomes. Pediatric transplantation Hollander, S. A., McElhinney, D. B., Almond, C. S., McDonald, N., Chen, S., Kaufman, B. D., Bernstein, D., Rosenthal, D. N. 2017; 21 (1)

    Abstract

    We report the patterns of rehospitalization after pediatric heart transplant (Htx) at a single center. Retrospective review of 107 consecutive pediatric Htx recipients between January 22, 2007, and August 28, 2014, who survived their initial transplant hospitalization. The frequency, duration, and indications for all hospitalizations between transplant hospitalization discharge and September 30, 2015, were analyzed. A total of 444 hospitalization episodes occurred in 90 of 107 (84%) patients. The median time to first rehospitalization was 59.5 (range 1-1526) days, and the median length of stay was 2.5 (range 0-81) days. There were an average of two hospitalizations per patient in the first year following transplant hospitalization, declining to about 0.8 per patient per year starting at 3 years post-transplant. Admissions for viral infections were most common, occurring in 93 of 386 (24%), followed by rule out sepsis in 61 of 386 (16%). Admissions for suspected or confirmed rejection were less frequent, accounting for 41 of 386 (11%) and 31 of 386 (8%) of all admissions, respectively. Survival to discharge after rehospitalization was 97%. Hospitalization is common after pediatric Htx, particularly in the first post-transplant year, with the most frequent indications for hospitalization being viral illness and rule out sepsis. After the first post-transplant year, the risk for readmission falls significantly but remains constant for several years.

    View details for DOI 10.1111/petr.12857

    View details for PubMedID 27891727

  • Transcatheter valve implantation for right atrium-to-right ventricle conduit obstruction or regurgitation after modified Björk-fontan procedure. Catheterization and cardiovascular interventions Shah, A. H., Horlick, E. M., Eicken, A., Asnes, J. D., Bocks, M. L., Boudjemline, Y., Cabalka, A. K., Fagan, T. E., Schubert, S., Mahadevan, V. S., Dvir, D., Osten, M., McElhinney, D. B. 2017; 89 (2): 298-305

    Abstract

    The most common reason for reintervention after a Björk modification of the Fontan procedure, in which the right ventricle (RV) is incorporated into the pulmonary circulation by connecting the right atrial (RA) appendage to the RV directly or with an extra-anatomic graft, is obstruction or regurgitation of the RA-RV connection. Transcatheter implantation of a valved stent is an appealing option for the treatment of RA-RV conduit dysfunction in these patients. In the present study, we assessed early and intermediate results after transcatheter valve implantation within an obstructed or regurgitant RA-RV modified Fontan pathway.Through a retrospective multicenter registry, we collected data from 16 patients with a modified Fontan circulation who were treated with percutaneous Melody or Sapien valve implantation for dysfunction of an extra-anatomic RA-RV conduit or valve.All patients had successful and uncomplicated implantation of a Melody (n = 15) or Sapien 3 (n = 1) valve with hemodynamic and, in most cases, clinical improvement. During a median follow-up of 3.3 years, 3 patients died of cardiovascular causes unrelated to the procedure or the valve, and no major valve dysfunction was observed.Percutaneous transcatheter valve deployment to treat a dysfunctional RA-RV connection after a Björk modification of the Fontan procedure is a viable alternative to surgery, with low procedural risk, and appears to offer good early and intermediate results.© 2016 Wiley Periodicals, Inc.

    View details for DOI 10.1002/ccd.26648

    View details for PubMedID 27465501

  • Reflection and Rationalization: Making Sense of the Literature on Endocarditis After Transcatheter Pulmonary Valve Replacement. Circulation. Cardiovascular interventions McElhinney, D. B. 2017; 10 (2)
  • Circulatory support using the impella device in fontan patients with systemic ventricular dysfunction: A multicenter experience. Catheterization and cardiovascular interventions Morray, B. H., Dimas, V. V., Lim, S., Balzer, D. T., Parekh, D. R., Van Mieghem, N. M., Ewert, P., Kim, D. W., Justino, H., McElhinney, D. B., Jones, T. K. 2017

    Abstract

    There are limited mechanical circulatory support options for patients with single ventricle (SV) anatomy. This is a multicenter, retrospective study of the Impella pump to support the systemic ventricle in a cohort of SV patients with Fontan circulation.Patients with SV anatomy supported with an Impella device from 2012 to 2015 were included. Demographic information, indication for support, adverse events and short-term outcome data were collected.Ten patients were included. The median age and weight at implant was 26 years (4-38 years) and 64 kg (15-102 kg). Indications for support were systemic ventricular failure with cardiogenic shock (n = 8) or high-risk electrophysiology (EP) procedures (n = 2). The median duration of support was 49 hr (2.7-264 hr). Support was discontinued for ventricular recovery in five patients, transition to another device in two patients, completion of EP procedure in two patients and death in one patient. Survival to hospital discharge was 80%. Adverse events occurred in 4 patients. There were two cases of hemolysis, one case of increasing aortic valve insufficiency with implant and one asymptomatic access site thrombus. There were no bleeding or thromboembolic events.Impella devices can provide temporary support for the systemic ventricle in SV patients as a bridge to recovery or additional device. Procedural survival and adverse event profiles are favorable. © 2017 Wiley Periodicals, Inc.

    View details for DOI 10.1002/ccd.26885

    View details for PubMedID 28112463

  • Transcatheter Valve-in-Ring Implantation for the Treatment of Residual or Recurrent Tricuspid Valve Dysfunction After Prior Surgical Repair JACC-CARDIOVASCULAR INTERVENTIONS Aboulhosn, J., Cabalka, A. K., Levi, D. S., Himbert, D., Testa, L., Latib, A., Makkar, R. R., Boudjemline, Y., Kim, D. W., Kefer, J., Bleiziffer, S., Kerst, G., Dvir, D., McElhinney, D. B. 2017; 10 (1): 53-63

    Abstract

    This study sought to describe the results of transcatheter tricuspid valve-in-ring (TVIR) implantation for treatment of tricuspid regurgitation (TR).Off-label use of transcatheter valves within surgically placed tricuspid annuloplasty prostheses has only been described in small reports. An international multicenter registry was developed to collect data on TVIR implantation.Data were collected from 13 sites on 22 patients (5 to 69 years of age) with TR who underwent catheterization with the intent to perform TVIR implantation.TVIR implantation was performed in 20 patients (91%). Most patients were severely impaired (86% in New York Heart Association functional class III or IV); TR was severe in 86%. A Sapien valve (Edwards Lifesciences, Irvine, California) was implanted in 17 patients and a Melody valve (Medtronic, Minneapolis, Minnesota) in 3. There were no procedural deaths. There was 1 valve embolization requiring retrieval and placement of second TVIR implant and 1 valve malposition with severe paravalvular regurgitation requiring a second TVIR implantation. Over a median follow-up of 12 months, 1 patient died and 2 underwent repeat TVIR implantation, 1 of whom subsequently underwent surgical valve replacement. Significant paravalvular leak (PVL) was treated at the time of TVIR implantation in 4 patients: 3 underwent device occlusion and 1 received a second TVIR implant. On follow-up echocardiography, 15 patients had PVL (75%), the majority of which (n = 10) were trivial or mild and did not require treatment. PVL intervention was performed in 3 patients during follow-up. Functional capacity improved in most patients (70%).TVIR implantation using commercially available transcatheter prostheses is technically feasible and clinically effective in reducing TR. Paravalvular regurgitation is common and may necessitate further interventions.

    View details for DOI 10.1016/j.jcin.2016.10.036

    View details for Web of Science ID 000392624100010

    View details for PubMedID 28057286

  • Risk Estimates for Atherosclerotic Cardiovascular Disease in Adults With Congenital Heart Disease AMERICAN JOURNAL OF CARDIOLOGY Lui, G. K., Rogers, I. S., Ding, V. Y., Hedlin, H. K., MacMillen, K., Maron, D. J., Sillman, C., Romfh, A., Dade, T. C., Haeffele, C., Grady, S. R., McElhinney, D. B., Murphy, D. J., Fernandes, S. M. 2017; 119 (1): 112-118

    Abstract

    The adult with congenital heart disease (CHD) is at risk of developing atherosclerotic cardiovascular disease (ASCVD). We performed a cross-sectional study to describe established ASCVD risk factors and estimate 10-year and lifetime risk of ASCVD in adults over age 18 with CHD of moderate or great complexity using 3 validated risk assessment tools-the Framingham Study Cardiovascular Disease Risk Assessment, the Reynolds Risk Score, and the ASCVD Risk Estimator. We obtained extensive clinical and survey data on 178 enrolled patients, with average age 37.1 ± 12.6 years, 51% men. At least 1 modifiable ASCVD risk factor was present in 70%; the 2 most common were overweight/obesity (53%) and systemic hypertension (24%). Laboratory data were available in 103 of the 178 patients. Abnormal levels of glycated hemoglobin, high-sensitivity C-reactive protein, and high-density lipoprotein were each found in around 30% of patients. The 10-year ASCVD predicted risk using all 3 tools was relatively low (i.e., at least 90% of patients <10% risk), yet the median estimated lifetime risk was 36%. In conclusion, ASCVD risk factors are prevalent in adults with CHD. The risk estimation tools suggest that this population is particularly vulnerable to ASCVD with aging and should undergo guideline-based screening and management of modifiable risk factors.

    View details for DOI 10.1016/j.amjcard.2016.09.023

    View details for Web of Science ID 000391246900018

    View details for PubMedID 28247847

    View details for PubMedCentralID PMC5334785

  • Utility and Scope of Rapid Prototyping in Patients with Complex Muscular Ventricular Septal Defects or Double-Outlet Right Ventricle: Does it Alter Management Decisions? Pediatric cardiology Bhatla, P., Tretter, J. T., Ludomirsky, A., Argilla, M., Latson, L. A., Chakravarti, S., Barker, P. C., Yoo, S., McElhinney, D. B., Wake, N., Mosca, R. S. 2017; 38 (1): 103-114

    Abstract

    Rapid prototyping facilitates comprehension of complex cardiac anatomy. However, determining when this additional information proves instrumental in patient management remains a challenge. We describe our experience with patient-specific anatomic models created using rapid prototyping from various imaging modalities, suggesting their utility in surgical and interventional planning in congenital heart disease (CHD). Virtual and physical 3-dimensional (3D) models were generated from CT or MRI data, using commercially available software for patients with complex muscular ventricular septal defects (CMVSD) and double-outlet right ventricle (DORV). Six patients with complex anatomy and uncertainty of the optimal management strategy were included in this study. The models were subsequently used to guide management decisions, and the outcomes reviewed. 3D models clearly demonstrated the complex intra-cardiac anatomy in all six patients and were utilized to guide management decisions. In the three patients with CMVSD, one underwent successful endovascular device closure following a prior failed attempt at transcatheter closure, and the other two underwent successful primary surgical closure with the aid of 3D models. In all three cases of DORV, the models provided better anatomic delineation and additional information that altered or confirmed the surgical plan. Patient-specific 3D heart models show promise in accurately defining intra-cardiac anatomy in CHD, specifically CMVSD and DORV. We believe these models improve understanding of the complex anatomical spatial relationships in these defects and provide additional insight for pre/intra-interventional management and surgical planning.

    View details for DOI 10.1007/s00246-016-1489-1

    View details for PubMedID 27837304

  • Practice Patterns in Postoperative Echocardiographic Surveillance after Congenital Heart Surgery in Children: A Single Center Experience JOURNAL OF PEDIATRICS Arunamata, A., Axelrod, D. M., Kipps, A. K., McElhinney, D. B., Shin, A. Y., Hanley, F. L., Olson, I. L., Roth, S. J., Tierney, E. S. 2017; 180: 87-?

    Abstract

    To review current institutional practice and describe factors contributing to variation in inpatient postoperative imaging surveillance after congenital heart surgery.We reviewed records of all children who underwent congenital heart surgery from June to December 2014. Number and primary indications for postoperative transthoracic echocardiograms (TTEs), providers involved, cardiovascular intensive care unit (CVICU) and total hospital length of stay, and Risk-Adjustment for Congenital Heart Surgery-1 scores were recorded.A total of 253 children (age at surgery: 8 months [2 days-19 years]) received 556 postoperative TTEs (median 1 TTE/patient [1-14]), and 23% had ≥3 TTEs. Fifteen of 556 TTEs (2.7%) revealed a new abnormal finding. The majority of TTEs (59%) were performed in the CVICU (1.5 ± 1.1 TTEs/week/patient), with evaluation of function as the most common indication (44%). Attending physician practice >10 years was not associated with fewer TTEs (P = .12). Patients with ≥3 TTEs had higher Risk-Adjustment for Congenital Heart Surgery-1 scores (P = .001), longer CVICU lengths of stay (22 vs 3 days; P < .0001), longer overall hospitalizations (28 vs 7 days; P < .0001), and a higher incidence of mechanical circulatory support (10% vs 0%; P < .0001) than those with <3 TTEs. Eight patients with ≥3 TTEs did not survive, compared with 3 with <3 TTEs (P = .0004).There was wide intra-institutional variation in echocardiographic use among similar complexity surgeries. Frequency of postoperative echocardiographic surveillance was associated with degree of surgical complexity and severity of postoperative clinical condition. Few studies revealed new abnormal findings. These results may help establish evidence-based guidelines for inpatient echocardiographic surveillance after congenital heart surgery.

    View details for DOI 10.1016/j.jpeds.2016.09.061

    View details for Web of Science ID 000390028100018

  • Assessment of Progressive Pathophysiology After Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia AMERICAN JOURNAL OF CARDIOLOGY Tierney, E. S., McElhinney, D. B., Freud, L. R., Tworetzky, W., Cuneo, B. F., Escobar-Diaz, M. C., Ikemba, C., Kalish, B. T., Komarlu, R., Levasseur, S. M., Puchalski, M. D., Satou, G. M., Silverman, N. H., Moon-Grady, A. J. 2017; 119 (1): 106-111

    Abstract

    In fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD), poor hemodynamic status is associated with worse neonatal outcome. It is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in the third trimester. We evaluated if echocardiographic indexes in EA/TVD fetuses presenting <24 weeks of gestation are reliable indicators of physiologic status later in pregnancy. This multicenter, retrospective study included 51 fetuses presenting at <24 weeks of gestation with EA/TVD and serial fetal echocardiograms ≥4 weeks apart. We designated the following as markers of poor outcome: absence of anterograde flow across the pulmonary valve, pulmonary valve regurgitation, cardiothoracic area ratio >0.48, left ventricular (LV) dysfunction, or tricuspid valve (TV) annulus Z-score >5.6. Median gestational age at diagnosis was 21 weeks (range, 18 to 24). Eighteen fetuses (35%) had no markers for poor hemodynamic status initially, whereas only 7 of these continued to have no markers of poor outcome in the third trimester. Nine of 27 fetuses (33%) with anterograde pulmonary blood flow on the first echocardiogram developed pulmonary atresia; 7 of 39 (18%) developed new pulmonary valve regurgitation. LV dysfunction was present in 2 (4%) patients at <24 weeks but in 14 (37%) later (p <0.001). The TV annulus Z-score and cardiothoracic area both increased from diagnosis to follow-up. In conclusion, progressive hemodynamic compromise was common in this cohort. Our study highlights that care must be taken in counseling before 24 weeks, as the absence of factors associated with poor outcome early in pregnancy may be falsely reassuring.

    View details for DOI 10.1016/j.amjcard.2016.09.022

    View details for Web of Science ID 000391246900017

  • Unique Molecular Patterns Uncovered in Kawasaki Disease Patients with Elevated Serum Gamma Glutamyl Transferase Levels: Implications for Intravenous Immunoglobulin Responsiveness PLOS ONE Wang, Y., Li, Z., Hu, G., Hao, S., Deng, X., Huang, M., Ren, M., Jiang, X., Kanegaye, J. T., Ha, K., Lee, J., Li, X., Jiang, X., Yu, Y., Tremoulet, A. H., Burns, J. C., Whitin, J. C., Shin, A. Y., Sylvester, K. G., McElhinney, D. B., Cohen, H. J., Ling, X. B. 2016; 11 (12)

    Abstract

    Resistance to intravenous immunoglobulin (IVIG) occurs in 10-20% of patients with Kawasaki disease (KD). The risk of resistance is about two-fold higher in patients with elevated gamma glutamyl transferase (GGT) levels. We sought to understand the biological mechanisms underlying IVIG resistance in patients with elevated GGT levels.We explored the association between elevated GGT levels and IVIG-resistance with a cohort of 686 KD patients (Cohort I). Gene expression data from 130 children with acute KD (Cohort II) were analyzed using the R square statistic and false discovery analysis to identify genes that were differentially represented in patients with elevated GGT levels with regard to IVIG responsiveness. Two additional KD cohorts (Cohort III and IV) were used to test the hypothesis that sialylation and GGT may be involved in IVIG resistance through neutrophil apoptosis.Thirty-six genes were identified that significantly explained the variations of both GGT levels and IVIG responsiveness in KD patients. After Bonferroni correction, significant associations with IVIG resistance persisted for 12 out of 36 genes among patients with elevated GGT levels and none among patients with normal GGT levels. With the discovery of ST6GALNAC3, a sialyltransferase, as the most differentially expressed gene, we hypothesized that sialylation and GGT are involved in IVIG resistance through neutrophil apoptosis. We then confirmed that in Cohort III and IV there was significantly less reduction in neutrophil count in IVIG non-responders.Gene expression analyses combining molecular and clinical datasets support the hypotheses that: (1) neutrophil apoptosis induced by IVIG may be a mechanism of action of IVIG in KD; (2) changes in sialylation and GGT level in KD patients may contribute synergistically to IVIG resistance through blocking IVIG-induced neutrophil apoptosis. These findings have implications for understanding the mechanism of action in IVIG resistance, and possibly for development of novel therapeutics.

    View details for DOI 10.1371/journal.pone.0167434

    View details for Web of Science ID 000392853100008

    View details for PubMedID 28002448

    View details for PubMedCentralID PMC5176264

  • Web-based Real-Time Case Finding for the Population Health Management of Patients With Diabetes Mellitus: A Prospective Validation of the Natural Language Processing-Based Algorithm With Statewide Electronic Medical Records. JMIR medical informatics Zheng, L., Wang, Y., Hao, S., Shin, A. Y., Jin, B., Ngo, A. D., Jackson-Browne, M. S., Feller, D. J., Fu, T., Zhang, K., Zhou, X., Zhu, C., Dai, D., Yu, Y., Zheng, G., Li, Y., McElhinney, D. B., Culver, D. S., Alfreds, S. T., Stearns, F., Sylvester, K. G., Widen, E., Ling, X. B. 2016; 4 (4)

    Abstract

    Diabetes case finding based on structured medical records does not fully identify diabetic patients whose medical histories related to diabetes are available in the form of free text. Manual chart reviews have been used but involve high labor costs and long latency.This study developed and tested a Web-based diabetes case finding algorithm using both structured and unstructured electronic medical records (EMRs).This study was based on the health information exchange (HIE) EMR database that covers almost all health facilities in the state of Maine, United States. Using narrative clinical notes, a Web-based natural language processing (NLP) case finding algorithm was retrospectively (July 1, 2012, to June 30, 2013) developed with a random subset of HIE-associated facilities, which was then blind tested with the remaining facilities. The NLP-based algorithm was subsequently integrated into the HIE database and validated prospectively (July 1, 2013, to June 30, 2014).Of the 935,891 patients in the prospective cohort, 64,168 diabetes cases were identified using diagnosis codes alone. Our NLP-based case finding algorithm prospectively found an additional 5756 uncodified cases (5756/64,168, 8.97% increase) with a positive predictive value of .90. Of the 21,720 diabetic patients identified by both methods, 6616 patients (6616/21,720, 30.46%) were identified by the NLP-based algorithm before a diabetes diagnosis was noted in the structured EMR (mean time difference = 48 days).The online NLP algorithm was effective in identifying uncodified diabetes cases in real time, leading to a significant improvement in diabetes case finding. The successful integration of the NLP-based case finding algorithm into the Maine HIE database indicates a strong potential for application of this novel method to achieve a more complete ascertainment of diagnoses of diabetes mellitus.

    View details for PubMedID 27836816

    View details for PubMedCentralID PMC5124114

  • Subacute left ventricular outflow tract obstruction after transapical closure of a mitral paravalvular leak in the region of the aortomitral curtain ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES Chikkabyrappa, S., McElhinney, D. B., Saric, M. 2016; 33 (11): 1771-1776

    Abstract

    We report a rare case of progressive left ventricular outflow tract (LVOT) obstruction after percutaneous device closure of a mechanical prosthetic mitral valve (MV) paravalvular leak (PVL) in the region of aortomitral curtain in a patient who also had small mechanical aortic valve prosthesis with patient-prosthesis mismatch.

    View details for DOI 10.1111/echo.13357

    View details for Web of Science ID 000388309500021

    View details for PubMedID 27576330

  • Practice Patterns in Postoperative Echocardiographic Surveillance after Congenital Heart Surgery in Children: A Single Center Experience. journal of pediatrics Arunamata, A., Axelrod, D. M., Kipps, A. K., McElhinney, D. B., Shin, A. Y., Hanley, F. L., Olson, I. L., Roth, S. J., Selamet Tierney, E. S. 2016

    Abstract

    To review current institutional practice and describe factors contributing to variation in inpatient postoperative imaging surveillance after congenital heart surgery.We reviewed records of all children who underwent congenital heart surgery from June to December 2014. Number and primary indications for postoperative transthoracic echocardiograms (TTEs), providers involved, cardiovascular intensive care unit (CVICU) and total hospital length of stay, and Risk-Adjustment for Congenital Heart Surgery-1 scores were recorded.A total of 253 children (age at surgery: 8 months [2 days-19 years]) received 556 postoperative TTEs (median 1 TTE/patient [1-14]), and 23% had ≥3 TTEs. Fifteen of 556 TTEs (2.7%) revealed a new abnormal finding. The majority of TTEs (59%) were performed in the CVICU (1.5 ± 1.1 TTEs/week/patient), with evaluation of function as the most common indication (44%). Attending physician practice >10 years was not associated with fewer TTEs (P = .12). Patients with ≥3 TTEs had higher Risk-Adjustment for Congenital Heart Surgery-1 scores (P = .001), longer CVICU lengths of stay (22 vs 3 days; P < .0001), longer overall hospitalizations (28 vs 7 days; P < .0001), and a higher incidence of mechanical circulatory support (10% vs 0%; P < .0001) than those with <3 TTEs. Eight patients with ≥3 TTEs did not survive, compared with 3 with <3 TTEs (P = .0004).There was wide intra-institutional variation in echocardiographic use among similar complexity surgeries. Frequency of postoperative echocardiographic surveillance was associated with degree of surgical complexity and severity of postoperative clinical condition. Few studies revealed new abnormal findings. These results may help establish evidence-based guidelines for inpatient echocardiographic surveillance after congenital heart surgery.

    View details for DOI 10.1016/j.jpeds.2016.09.061

    View details for PubMedID 28029346

  • Defining and refining indications for transcatheter pulmonary valve replacement in patients with repaired tetralogy of Fallot: Contributions from anatomical and functional imaging. International journal of cardiology Tretter, J. T., Friedberg, M. K., Wald, R. M., McElhinney, D. B. 2016; 221: 916-925

    Abstract

    Transcatheter pulmonary valve replacement (TPVR) is an important treatment option in repaired tetralogy of Fallot (TOF) and right ventricular outflow tract (RVOT) dysfunction. Indications for timing of TPVR are extrapolated from surgical pulmonary valve replacement guidelines, which are themselves controversial as published evidence is scarce and expert opinion therefore prevails. We review current indications for PVR following TOF repair, focusing on those for TPVR specifically, and discuss anatomical and functional considerations as these pertain to determination of candidacy for TPVR. Hemodynamic assessment surrounding PVR has focused on assessment of the right ventricle (RV) size and systolic function, with the goal of intervening in the asymptomatic patient prior to the development of irreversible RV deterioration and right heart failure. The impact of abnormal RV mechanics on the LV has been appreciated, with the assessment of LV function assuming higher priority in decision-making regarding possible PVR. In addition to the standard volumetric assessment, evaluation with indices of myocardial wall strain, tissue velocities, diastology, and ventricular response to exercise is emerging as tools with potential to further refine timing of PVR. We conclude that, at present, current evidence, although limited, supports a more aggressive approach in those who meet inclusion for TPVR in patients with repaired TOF and RVOT dysfunction guided by the discussed hemodynamic assessment, however, more importantly this review should lay the framework for future investigations regarding hemodynamic assessment of this population.

    View details for DOI 10.1016/j.ijcard.2016.07.120

    View details for PubMedID 27441469

  • Transcatheter Pulmonary Valve Replacement Reduces Tricuspid Regurgitation in Patients With Right Ventricular Volume/Pressure Overload. Journal of the American College of Cardiology Jones, T. K., Rome, J. J., Armstrong, A. K., Berger, F., Hellenbrand, W. E., Cabalka, A. K., Benson, L. N., Balzer, D. T., Cheatham, J. P., Eicken, A., McElhinney, D. B. 2016; 68 (14): 1525-1535

    Abstract

    Tricuspid regurgitation (TR) is a common and important comorbidity in patients with postoperative right ventricular outflow tract (RVOT) obstruction or pulmonary regurgitation (PR). Transcatheter pulmonary valve replacement (TPVR) has become a useful tool in the management of postoperative RVOT obstruction and PR, but it is unknown whether relief of the right ventricular volume and/or pressure overload by TPVR will have a beneficial effect on TR, as is often seen with surgical pulmonary valve replacement.This study sought to assess the prevalence of and factors associated with significant TR in patients undergoing TPVR for RVOT obstruction or PR.Data were combined from 3 prospective multicenter trials of patients referred for TPVR. Follow-up data through 5 years post-implantation were analyzed.Of 300 patients studied, 77 (25.6%) had moderate or severe TR at baseline. After TPVR, TR severity was improved in 65% of those patients, and more than one-half had mild TR or less TR at discharge. Of 13 patients with severe TR pre-implantation, only 1 had severe TR at 1-year follow-up and beyond. Moderate or severe baseline TR was associated with shorter freedom from RVOT reintervention after TPVR.In this prospective multicenter study of post-operative patients with RVOT obstruction and/or PR, TR was common. In patients with significant baseline TR, TPVR resulted in clinically relevant acute reductions in TR that persisted over at least 5 years of follow-up. These observations support the application of TPVR therapy in patients with RVOT obstruction or PR who are anatomically suitable, even in the setting of significant concomitant TR.

    View details for DOI 10.1016/j.jacc.2016.07.734

    View details for PubMedID 27687194

  • Assessment of Progressive Pathophysiology After Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia. American journal of cardiology Selamet Tierney, E. S., McElhinney, D. B., Freud, L. R., Tworetzky, W., Cuneo, B. F., Escobar-Diaz, M. C., Ikemba, C., Kalish, B. T., Komarlu, R., Levasseur, S. M., Puchalski, M. D., Satou, G. M., Silverman, N. H., Moon-Grady, A. J. 2016

    Abstract

    In fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD), poor hemodynamic status is associated with worse neonatal outcome. It is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in the third trimester. We evaluated if echocardiographic indexes in EA/TVD fetuses presenting <24 weeks of gestation are reliable indicators of physiologic status later in pregnancy. This multicenter, retrospective study included 51 fetuses presenting at <24 weeks of gestation with EA/TVD and serial fetal echocardiograms ≥4 weeks apart. We designated the following as markers of poor outcome: absence of anterograde flow across the pulmonary valve, pulmonary valve regurgitation, cardiothoracic area ratio >0.48, left ventricular (LV) dysfunction, or tricuspid valve (TV) annulus Z-score >5.6. Median gestational age at diagnosis was 21 weeks (range, 18 to 24). Eighteen fetuses (35%) had no markers for poor hemodynamic status initially, whereas only 7 of these continued to have no markers of poor outcome in the third trimester. Nine of 27 fetuses (33%) with anterograde pulmonary blood flow on the first echocardiogram developed pulmonary atresia; 7 of 39 (18%) developed new pulmonary valve regurgitation. LV dysfunction was present in 2 (4%) patients at <24 weeks but in 14 (37%) later (p <0.001). The TV annulus Z-score and cardiothoracic area both increased from diagnosis to follow-up. In conclusion, progressive hemodynamic compromise was common in this cohort. Our study highlights that care must be taken in counseling before 24 weeks, as the absence of factors associated with poor outcome early in pregnancy may be falsely reassuring.

    View details for DOI 10.1016/j.amjcard.2016.09.022

    View details for PubMedID 27793395

  • Progressive intermediate-term improvement in ventricular and atrioventricular interaction after transcatheter pulmonary valve replacement in patients with right ventricular outflow tract obstruction. American heart journal Lunze, F. I., Hasan, B. S., Gauvreau, K., Brown, D. W., Colan, S. D., McElhinney, D. B. 2016; 179: 87-98

    Abstract

    Relief of postoperative right ventricular outflow tract (RVOT) obstruction with transcatheter pulmonary valve replacement (TPVR) results in functional improvement in the short term which we investigated at baseline (BL), early follow-up (FU), and midterm FU after TPVR.Echocardiography and cardiopulmonary exercise testing were performed at BL and at early (median 6 months) and midterm FU (median 2.5years) after TPVR.Patients with RVOT obstruction (n=22, median age 17years) were studied. The max RVOT Doppler gradient fell from BL to early FU (60±24 to 26±8mm Hg, P<.001). Left ventricular (LV) end-diastolic and stroke volume increased at early FU (both P<.001) without further change, whereas LV ejection fraction improved throughout FU (P<.001). LV end-systolic and diastolic eccentricity (leftward septal displacement) improved early (both P≤.003), and end-diastolic eccentricity improved further at midterm FU (P=.02). Furthermore, whereas mitral inflow A wave velocity increased (P=.003), the LV A' velocity declined early (P=.007) without further change at midterm. RV systolic and early diastolic function was impaired at BL. Whereas RV strain improved partially at early and midterm FU (P≤.02), RV E' velocity did not improve throughout FU. Mildly impaired LV strain at BL fully recovered by midterm FU (P≤.002). Peak oxygen uptake improved at early and midterm FU (all P≤.003).Patients with RVOT obstruction had biventricular systolic and diastolic dysfunction at BL. Relieving RVOT obstruction with TPVR reduced adverse ventricular and compensatory atrioventricular interaction, resulting in progressive biventricular functional improvement and remodeling at early and midterm FU.

    View details for DOI 10.1016/j.ahj.2016.05.011

    View details for PubMedID 27595683

  • Sequential Percutaneous Closure of Mitral Prosthetic Paravalvular Leak and Complex Communicating Pseudoaneurysms of the Ascending Aorta and Subvalvar Left Ventricular Outflow Tract CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Tretter, J. T., Latson, L. A., McElhinney, D. B. 2016; 88 (1): 150-156

    Abstract

    Ascending aortic and subvalvar left ventricular outflow tract (LVOT) pseudoaneurysms are rare complications following aortic valve or root replacement surgery. Clinically important paravalvular leaks are rare complications following any valve replacement surgery. We report an unusual case of sequential percutaneous closure of mitral prosthetic paravalvular leak and complex communicating ascending aortic and subvalvar LVOT pseudoaneurysms, which demonstrates the importance of multimodal imaging assessment surrounding percutaneous closure. © 2015 Wiley Periodicals, Inc.

    View details for DOI 10.1002/ccd.26001

    View details for Web of Science ID 000379984000031

    View details for PubMedID 25964108

  • Postoperative Outcomes of Children With Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals Undergoing Reconstruction of Occluded Pulmonary Artery Branches ANNALS OF THORACIC SURGERY Asija, R., Koth, A. M., Velasquez, N., Chan, F. P., Perry, S. B., Hanley, F. L., McElhinney, D. 2016; 101 (6): 2329-2334

    Abstract

    Patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/PA/MAPCAs) undergoing unifocalization surgery are at risk for prolonged postoperative respiratory failure. We sought to understand whether patients undergoing reconstruction and incorporation of occluded pulmonary arterial branches were at risk for worse postoperative outcomes.We performed a retrospective chart review to identify patients who underwent unifocalization or unifocalization revision with incorporation of occluded pulmonary artery branches. Patients with and without occluded branches were compared, with a focus on clinical outcomes.We studied 92 patients who underwent unifocalization procedures between 2010 and 2014, 17 (18%) of whom underwent reconstruction of occluded pulmonary artery branches. Patients with occluded vessels were more likely to require staged unifocalization procedures, although more than two thirds of this cohort eventually underwent complete intracardiac repair. Durations of mechanical ventilation, intensive care, hospital stay, and the need for early reoperation were similar between the two groups.Occluded pulmonary arterial branches can be safely recruited into the pulmonary vasculature in patients with TOF/PA/MAPCAs without a significant difference in postoperative outcomes compared with patients who did not have an occluded branch. Incorporation of occluded branches may also facilitate ultimate complete intracardiac repair in this complex population of patients.

    View details for DOI 10.1016/j.athoracsur.2015.12.049

    View details for Web of Science ID 000376502600048

    View details for PubMedID 26947013

  • Identifying Gaps in Technology for Congenital Interventions: Analysis of a Needs Survey from Congenital Interventional Cardiologists PEDIATRIC CARDIOLOGY Shibbani, K., Kenny, D., McElhinney, D., Hijazi, Z. M., Moran, T. 2016; 37 (5): 925-931

    Abstract

    We carried out a device-needs survey to evaluate the gaps in device and equipment availability for congenital interventional cardiologists. As the complexity and demand for more complete solutions to congenital heart lesions increase, there is a growing need for modification and development of devices and equipment to support this endeavor. The survey was sent out via e-mail to members of the Congenital Cardiovascular Interventional Study Consortium and the Society for Cardiac Angiography and Interventions with a reach of over 350 congenital interventionalists. Responses were received from 68 cardiologists in 8 countries. In terms of the most desired device, 41 % ranked bioresorbable stents as their first choice from a list of 12 possible devices. Similarly, 23 % ranked large covered stents as their first choice. Twenty-seven percent of participants believed bioresorbable stents would have the greatest potential to improve morbidity of their patients, with another 27 % reporting that covered stents would have the greatest impact. Fifty percent of participants reported that they would like to see large covered stents available in their country. These data point toward a perceived need for the development/approval of bioresorbable stents for the pediatric age group, as well as the need to approve the use of large covered stents in the pediatric age group in the USA.

    View details for DOI 10.1007/s00246-016-1372-0

    View details for Web of Science ID 000377722400016

    View details for PubMedID 27064094

  • Retrograde Transcatheter Device Closure of a Complex Paravalvular Leak After Bioprosthetic Pulmonary Valve Replacement in a Pediatric Patient CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Chikkabyrappa, S., Mosca, R. S., McElhinney, D. B. 2016; 87 (7): 1294-1297

    Abstract

    We report a case of retrograde transcatheter device closure of a complex paravalvular leak (PVL) after bioprosthetic pulmonary valve replacement (PVR) in a 13-year-old patient with congenital pulmonary valve stenosis. There are prior reports of pulmonary PVL closure after PVR in adults (Seery and Slack, Congenit Heart Dis 2014;9:E19-F22), but indications for and technical considerations in PVL closure after bioprosthetic PVR, particularly in children, are not well defined. © 2015 Wiley Periodicals, Inc.

    View details for DOI 10.1002/ccd.26316

    View details for Web of Science ID 000379985100023

    View details for PubMedID 26514545

  • Relative Risk Factors for Cardiac Erosion Following Transcatheter Closure of Atrial Septal Defects A Case-Control Study CIRCULATION McElhinney, D. B., Quartermain, M. D., Kenny, D., Alboliras, E., Amin, Z. 2016; 133 (18): 1738-?

    Abstract

    Transcatheter closure of secundum atrial septal defects (ASD) using the Amplatzer septal occluder is generally safe and effective, but erosion into the pericardial space or aorta has been described. Although the absolute risk of this complication is low, there has been no assessment of relative risk factors.All erosions reported to St. Jude Medical after ASD closure with an Amplatzer septal occluder (cases) were compared with controls (matched 2:1) who underwent ASD closure but did not develop an erosion. A total of 125 erosions were reported between 2002 and 2014, including 95 with an available echocardiogram. The median duration from implant to erosion was 14 days, but was >1 year in 16 patients. Nine patients (all age ≥17 years) who died were more likely to have an oversized device, and to have erosion into the aorta, than survivors. Aortic or superior vena cava rim deficiencies were more common in cases than in controls. In addition, larger balloon-sized ASD diameter, Amplatzer septal occluder device size, and device size-ASD diameter difference, and smaller weight:device size ratio were associated with erosion. On multivariable analysis, deficiency of any rim, device >5 mm larger than ASD diameter, and weight:device size ratio were associated with erosion.In addition to aortic rim deficiency, which was almost universal among erosion cases, there were several relative risk factors for erosion after ASD closure with the Amplatzer septal occluder device. To understand the mechanisms of and absolute risk factors for this uncommon but serious complication, an adequately powered prospective study with thorough echocardiographic evaluation will be critical.

    View details for DOI 10.1161/CIRCULATIONAHA.115.019987

    View details for Web of Science ID 000375604400004

    View details for PubMedID 27002094

  • Transcatheter Tricuspid Valve-in-Valve Implantation for the Treatment of Dysfunctional Surgical Bioprosthetic Valves An International, Multicenter Registry Study CIRCULATION McElhinney, D. B., Cabalka, A. K., AboulHosn, J. A., Eicken, A., Boudjemline, Y., Schubert, S., Himbert, D., Asnes, J. D., Salizzoni, S., Bocks, M. L., Cheatham, J. P., Momenah, T. S., Kim, D. W., Schranz, D., Meadows, J., Thomson, J. D., Goldstein, B. H., Crittendon, I., Fagan, T. E., Webb, J. G., Horlick, E., Delaney, J. W., Jones, T. K., Shahanavaz, S., Moretti, C., Hainstock, M. R., Kenny, D. P., Berger, F., Rihal, C. S., Dvir, D. 2016; 133 (16): 1582-?

    Abstract

    Off-label use of transcatheter aortic and pulmonary valve prostheses for tricuspid valve-in-valve implantation (TVIV) within dysfunctional surgical tricuspid valve (TV) bioprostheses has been described in small reports.An international, multicenter registry was developed to collect data on TVIV cases. Patient-related factors, procedural details and outcomes, and follow-up data were analyzed. Valve-in-ring or heterotopic TV implantation procedures were not included. Data were collected on 156 patients with bioprosthetic TV dysfunction who underwent catheterization with planned TVIV. The median age was 40 years, and 71% of patients were in New York Heart Association class III or IV. Among 152 patients in whom TVIV was attempted with a Melody (n=94) or Sapien (n=58) valve, implantation was successful in 150, with few serious complications. After TVIV, both the TV inflow gradient and tricuspid regurgitation grade improved significantly. During follow-up (median, 13.3 months), 22 patients died, 5 within 30 days; all 22 patients were in New York Heart Association class III or IV, and 9 were hospitalized before TVIV. There were 10 TV reinterventions, and 3 other patients had significant recurrent TV dysfunction. At follow-up, 77% of patients were in New York Heart Association class I or II (P<0.001 versus before TVIV). Outcomes did not differ according to surgical valve size or TVIV valve type.TVIV with commercially available transcatheter prostheses is technically and clinically successful in patients of various ages across a wide range of valve size. Although preimplantation clinical status was associated with outcome, many patients in New York Heart Association class III or IV at baseline improved. TVIV should be considered a viable option for treatment of failing TV bioprostheses.

    View details for DOI 10.1161/CIRCULATIONAHA.115.019353

    View details for Web of Science ID 000374553400013

    View details for PubMedID 26994123

  • Intentional Fracture of Maximally Dilated Balloon-Expandable Pulmonary Artery Stents Using Ultra-High-Pressure Balloon Angioplasty: A Preliminary Analysis. Circulation. Cardiovascular interventions Morray, B. H., McElhinney, D. B., Marshall, A. C., Porras, D. 2016; 9 (4)

    Abstract

    Treatment with endovascular stents has become increasingly common for the management of vascular stenosis in congenital heart disease. The use of stents in smaller patients has been tempered by concerns about the potential for stent expansion to accommodate somatic growth. One solution to limited stent diameter is the intentional fracture of maximally dilated stents, which can be accomplished using ultra-high-pressure (UHP) balloons.This retrospective cohort study compared procedural characteristics and adverse events between a cohort of patients with branch pulmonary artery (PA) stents who underwent stent fracture using UHP balloons and control patients who underwent UHP redilation of previously placed PA stents without stent fracture between 2004 and 2014. Two control patients were selected for every case. Thirty-three PA stents were fractured in 31 patients with a median of 10 years after initial stent placement. The median balloon:waist ratio was 1.17 (1-1.71), and the median inflation pressure was 20 (8-30) atm. There were significant reductions in pressure gradient after angioplasty, with no difference in postangioplasty gradients between cases and controls. There were no major PA complications in the stent fracture group and no difference in the number of adverse events between the 2 groups.In this small series, PA stent fracture using UHP balloon angioplasty was feasible and did not result in major complications although predictors of successful fracture were not identified. Intentional fracture with UHP balloon angioplasty may be considered when treating stents that have become restrictive despite maximal dilation.

    View details for DOI 10.1161/CIRCINTERVENTIONS.115.003281

    View details for PubMedID 27059684

  • Aortic Root Distortion and Aortic Insufficiency During Balloon Angioplasty of the Right Ventricular Outflow Tract Prior to Transcatheter Pulmonary Valve Replacement. Journal of interventional cardiology Torres, A. J., McElhinney, D. B., Anderson, B. R., Turner, M. E., Crystal, M. A., Timchak, D. M., Vincent, J. A. 2016; 29 (2): 197-207

    Abstract

    To describe the significance of aortic root distortion (AD) and/or aortic valve insufficiency (AI) during balloon angioplasty of the right ventricular outflow tract (RVOT) performed to rule out coronary artery compression prior to transcatheter pulmonary valve (TPV) implantation.AD/AI was assessed by retrospective review of all procedural aortographies performed to evaluate coronary anatomy prior to TPV implantation. AD/AI was also reviewed in all pre-post MPV implant echocardiograms to assess for progression.From 04/2007 to 3/2015, 118 pts underwent catheterization with intent for TPV implant. Mean age and weight were 24.5 ± 12 years and 64.3 ± 20 kg, respectively. Diagnoses were: TOF (53%), D-TGA/DORV (18%), s/p Ross (15%), and Truncus (9%). Types of RV-PA connections were: conduits (96), bioprosthetic valves (14), and other (7). Successful TPV implant occurred in 91 pts (77%). RVOT balloon angioplasty was performed in 43/118 pts (36%). Aortography was performed in 18/43 pts with AD/AI noted in 6/18 (33%); 2 with D-TGA (1 s/p Lecompte, 1 s/p Rastelli), 2 with TOF, 1 Truncus and 1 s/p Ross. Procedure was aborted in the 2 who developed severe AD/AI. TPV was implanted in 3/4 patients with mild AD/AI. Review of pre-post TPV implantation echocardiograms in 83/91 pts (91%) revealed no new/worsened AI in any patient.AD/AI is relatively common on aortography during simultaneous RVOT balloon angioplasty. Lack of AI progression by echocardiography post-TPV implant suggests these may be benign findings in most cases. However, AD/AI should be carefully evaluated in certain anatomic subtypes with close RVOT/aortic alignments. (J Interven Cardiol 2016;29:197-207).

    View details for DOI 10.1111/joic.12270

    View details for PubMedID 26822282

  • Exploring the Role of Polycythemia in Patients With Cyanosis After Palliative Congenital Heart Surgery. Pediatric critical care medicine Siehr, S. L., Shi, S., Hao, S., Hu, Z., Jin, B., Hanley, F., Reddy, V. M., McElhinney, D. B., Ling, X. B., Shin, A. Y. 2016; 17 (3): 216-222

    Abstract

    To understand the relationship between polycythemia and clinical outcome in patients with hypoplastic left heart syndrome following the Norwood operation.A retrospective, single-center cohort study.Pediatric cardiovascular ICU, university-affiliated children's hospital.Infants with hypoplastic left heart syndrome admitted to our medical center from September 2009 to December 2012 undergoing stage 1/Norwood operation.None.Baseline demographic and clinical information including first recorded postoperative hematocrit and subsequent mean, median, and nadir hematocrits during the first 72 hours postoperatively were recorded. The primary outcomes were in-hospital mortality and length of hospitalization. Thirty-two patients were included in the analysis. Patients did not differ by operative factors (cardiopulmonary bypass time and cross-clamp time) or traditional markers of severity of illness (vasoactive inotrope score, lactate, saturation, and PaO2/FIO2 ratio). Early polycythemia (hematocrit value > 49%) was associated with longer cardiovascular ICU stay (51.0 [± 38.6] vs 21.4 [± 16.2] d; p < 0.01) and total hospital length of stay (65.0 [± 46.5] vs 36.1 [± 20.0] d; p = 0.03). In a multivariable analysis, polycythemia remained independently associated with the length of hospitalization after controlling for the amount of RBC transfusion (weight, 4.36 [95% CI, 1.35-7.37]; p < 0.01). No difference in in-hospital mortality rates was detected between the two groups (17.6% vs 20%).Early polycythemia following the Norwood operation is associated with longer length of hospitalization even after controlling for blood cell transfusion practices. We hypothesize that polycythemia may be caused by hemoconcentration and used as an early marker of capillary leak syndrome.

    View details for DOI 10.1097/PCC.0000000000000654

    View details for PubMedID 26825044

  • Exploring Value in Congenital Heart Disease: An Evaluation of Inpatient Admissions. Congenital heart disease Shin, A. Y., Hu, Z., Jin, B., Lal, S., Rosenthal, D. N., Efron, B., Sharek, P. J., Sutherland, S. M., Cohen, H. J., McElhinney, D. B., Roth, S. J., Ling, X. B. 2015; 10 (6): E278-87

    Abstract

    Understanding value provides an important context for improvement. However, most health care models fail to measure value. Our objective was to categorize inpatient encounters within an academic congenital heart program based on clinical outcome and the cost to achieve the outcome (value). We aimed to describe clinical and nonclinical features associated with value.We defined hospital encounters based on outcome per resource utilized. We performed principal component and cluster analysis to classify encounters based on mortality, length of stay, hospital cost and revenue into six classes. We used nearest shrunken centroid to identify discriminant features associated with the cluster-derived classes. These features underwent hierarchical clustering and multivariate analysis to identify features associated with each class.We analyzed all patients admitted to an academic congenital heart program between September 1, 2009, and December 31, 2012.A total of 2658 encounters occurred during the study period. Six classes were categorized by value. Low-performing value classes were associated with greater institutional reward; however, encounters with higher-performing value were associated with a loss in profitability. Encounters that included insertion of a pediatric ventricular assist device (log OR 2.5 [95% CI, 1.78 to 3.43]) and acquisition of a hospital-acquired infection (log OR 1.42 [95% CI, 0.99 to 1.87]) were risk factors for inferior health care value.Among the patients in our study, institutional reward was not associated with value. We describe a framework to target quality improvement and resource management efforts that can benefit patients, institutions, and payers alike.

    View details for DOI 10.1111/chd.12290

    View details for PubMedID 26219731

  • Exploring Value in Congenital Heart Disease: An Evaluation of Inpatient Admissions CONGENITAL HEART DISEASE Shin, A. Y., Hu, Z., Jin, B., Lal, S., Rosenthal, D. N., Efron, B., Sharek, P. J., Sutherland, S. M., Cohen, H. J., McElhinney, D. B., Roth, S. J., Ling, X. B. 2015; 10 (6): E278-E287

    Abstract

    Understanding value provides an important context for improvement. However, most health care models fail to measure value. Our objective was to categorize inpatient encounters within an academic congenital heart program based on clinical outcome and the cost to achieve the outcome (value). We aimed to describe clinical and nonclinical features associated with value.We defined hospital encounters based on outcome per resource utilized. We performed principal component and cluster analysis to classify encounters based on mortality, length of stay, hospital cost and revenue into six classes. We used nearest shrunken centroid to identify discriminant features associated with the cluster-derived classes. These features underwent hierarchical clustering and multivariate analysis to identify features associated with each class.We analyzed all patients admitted to an academic congenital heart program between September 1, 2009, and December 31, 2012.A total of 2658 encounters occurred during the study period. Six classes were categorized by value. Low-performing value classes were associated with greater institutional reward; however, encounters with higher-performing value were associated with a loss in profitability. Encounters that included insertion of a pediatric ventricular assist device (log OR 2.5 [95% CI, 1.78 to 3.43]) and acquisition of a hospital-acquired infection (log OR 1.42 [95% CI, 0.99 to 1.87]) were risk factors for inferior health care value.Among the patients in our study, institutional reward was not associated with value. We describe a framework to target quality improvement and resource management efforts that can benefit patients, institutions, and payers alike.

    View details for DOI 10.1111/chd.12290

    View details for Web of Science ID 000367379300004

  • Iatrogenic aortopulmonary communications after transcatheter interventions on the right ventricular outflow tract or pulmonary artery: Pathophysiologic, diagnostic, and management considerations CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Torres, A., Sanders, S. P., Vincent, J. A., El-Said, H. G., Leahy, R. A., Padera, R. F., McElhinney, D. B. 2015; 86 (3): 438-452

    Abstract

    To investigate the spectrum, etiology, and management of traumatic aortopulmonary (AP) communications after transcatheter interventions on the pulmonary circulation.An iatrogenic AP communication is an unusual complication after balloon pulmonary artery (PA) angioplasty or stenting, or transcatheter pulmonary valve replacement (TPVR). However, with the increasing application of transcatheter therapies for postoperative PA stenosis and right ventricular outflow tract (RVOT) dysfunction, including percutaneous pulmonary valve replacement, consideration of the etiology, diagnosis, and management of this problem is important for interventional cardiologists performing such procedures.We present three new cases, as well as gross anatomy and histopathology data, related to AP communications after PA interventions. We also review the literature relevant to this topic. Including these new cases, there have been 18 reported cases of iatrogenic AP communication after transcatheter interventions on the PAs or RVOT, primarily patients with transposition of the great arteries who underwent PA angioplasty after an arterial switch operation, or after TPVR in patients who had undergone a Ross procedure. The likely cause of such defects is PA trauma plus distortion of the neo-aortic anastomosis resulting from angioplasty or stenting of the RVOT or central PAs, with subsequent dissection through the extravascular connective tissue and into the closely adjacent vessel through the devitalized tissue at the anastomosis.Cardiologists performing PA or RVOT interventions should be aware of the possibility of a traumatic AP communication and consider this diagnosis when confronted with suggestive signs and symptoms. © 2015 Wiley Periodicals, Inc.

    View details for DOI 10.1002/ccd.25897

    View details for Web of Science ID 000359811600022

    View details for PubMedID 25676815

  • Aortic Wall Injury Related to Endovascular Therapy for Aortic Coarctation CIRCULATION-CARDIOVASCULAR INTERVENTIONS Tretter, J. T., Jones, T. K., McElhinney, D. B. 2015; 8 (9)
  • Transcatheter Pulmonary Valve Replacement for Right Ventricular Outflow Tract Conduit Dysfunction After the Ross Procedure ANNALS OF THORACIC SURGERY Gillespie, M. J., McElhinney, D. B., Kreutzer, J., Hellenbrand, W. E., El-Said, H., Ewert, P., Rhodes, J. F., Sondergaard, L., Jones, T. K. 2015; 100 (3): 996-1003
  • Transcatheter Pulmonary Valve Replacement for Right Ventricular Outflow Tract Conduit Dysfunction After the Ross Procedure. Annals of thoracic surgery Gillespie, M. J., McElhinney, D. B., Kreutzer, J., Hellenbrand, W. E., El-Said, H., Ewert, P., Rhodes, J. F., Søndergaard, L., Jones, T. K. 2015; 100 (3): 996-1002

    Abstract

    Right ventricular outflow tract (RVOT) conduit dysfunction is a limitation of the Ross procedure. Transcatheter pulmonary valve replacement (TPVR) could alter the impact of conduit dysfunction and the risk-benefit balance for the Ross procedure.Retrospective review of databases from 3 prospective Melody TPV (Medtronic Inc, Minneapolis, MN) trials.Among 358 patients who were catheterized with the intent to implant a Melody TPV for RVOT conduit stenosis or regurgitation (PR) as part of 3 prospective multicenter studies, 67 (19%) had a prior Ross procedure. Of these, 56 (84%) received a Melody valve; in 5 of the 11 patients who did not, the implant was aborted due to concern for coronary artery compression, and 1 implanted patient required emergent surgery for left coronary compression. The RVOT gradient decreased from a median 38 mm Hg to 13.5 mm Hg (p < 0.001). There was no or trivial PR in all but 4 patients, in whom it was mild. At a median follow-up of 4.0 years, 1 patient died from sepsis. Twelve patients underwent 14 transcatheter (n = 8) or surgical (n = 6) TPV reinterventions for obstruction with stent fracture (n = 9), endocarditis with conduit obstruction (n = 3), or reoperation (n = 2). Freedom from TPV explant was 89% ± 5% at 4 years. Among patients who did not undergo reintervention for obstruction, there was no change in RVOT gradient over time, and all but 1 patient had mild or less PR at last follow-up.The TPVR with the Melody valve provides acceptable early outcomes and durable valve function in the majority of Ross patients. Recurrent RVOT obstruction associated with stent fracture was the main reason for reintervention. Coronary compression is not uncommon in Ross patients and should be assessed prior to TPVR.

    View details for DOI 10.1016/j.athoracsur.2015.04.108

    View details for PubMedID 26190388

  • Aortic Wall Injury Related to Endovascular Therapy for Aortic Coarctation. Circulation. Cardiovascular interventions Tretter, J. T., Jones, T. K., McElhinney, D. B. 2015; 8 (9)

    Abstract

    Aortic wall complications can occur in unrepaired aortic coarctation (CoA) and after surgical repair or endovascular treatment. This review summarizes the available literature and current understanding of aortic wall injury (AWI) surrounding the management of CoA, focusing specifically on acute and follow-up AWI after endovascular treatment. There have been 23 reported cases of aortic rupture after endovascular treatment for CoA, including angioplasty alone, bare metal stenting, and primary covered stent therapy. Even if these published cases represent only a minority of ruptures that have actually occurred, the incidence is substantially <1%. The incidence of acute aneurysm formation was 0% to 13% after angioplasty, 0% to 5% after bare metal stent placement, and <1% after covered stent placement. The reported incidence and natural history of both acute and new AWI during follow-up after endovascular therapy for CoA varies considerably, likely secondary to ascertainment and reporting biases and inconsistent definitions. Although important AWI after endovascular treatment of CoA seems to be declining in frequency with increasing experience and improving technology, it remains one of the most important potential adverse outcomes. Long-term surveillance for new AWI and monitoring of existing AWI is mandatory, with institution of appropriate treatment when necessary. A central research focus in this population should be determination of the appropriate treatment for both native and recurrent CoA across various ages with regard to limiting recurrent CoA and preventing associated aortic wall complications, in addition to determining the appropriate treatment of various AWI. Consistent definitions and reporting are necessary to truly understand the incidence of, risk factors for, and measures protective against AWI after angioplasty or stent implantation for CoA.

    View details for DOI 10.1161/CIRCINTERVENTIONS.115.002840

    View details for PubMedID 26291468

  • Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era A Multicenter Study CIRCULATION Freud, L. R., Escobar-Diaz, M. C., Kalish, B. T., Komarlu, R., Puchalski, M. D., Jaeggi, E. T., Szwast, A. L., Freire, G., Levasseur, S. M., Kavanaugh-McHugh, A., Michelfelder, E. C., Moon-Grady, A. J., Donofrio, M. T., Howley, L. W., Tierney, E. S., Cuneo, B. F., Morris, S. A., Pruetz, J. D., van der Velde, M. E., Kovalchin, J. P., Ikemba, C. M., Vernon, M. M., Samai, C., Satou, G. M., Gotteiner, N. L., Phoon, C. K., Silverman, N. H., McElhinney, D. B., Tworetzky, W. 2015; 132 (6): 481-489

    Abstract

    Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era.Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1-6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001).In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

    View details for DOI 10.1161/CIRCULATIONAHA.115.015839

    View details for Web of Science ID 000359666500003

    View details for PubMedID 26059011

  • Preliminary Assessment of Tricuspid Valve Annular Velocity Parameters by Cardiac Magnetic Resonance Imaging in Adults with a Volume-Overloaded Right Ventricle: Comparison of Unrepaired Atrial Septal Defect and Repaired Tetralogy of Fallot PEDIATRIC CARDIOLOGY Ito, S., McElhinney, D. B., Adams, R., Bhatla, P., Chung, S., Axel, L. 2015; 36 (6): 1294-1300

    Abstract

    The aim is to compare tricuspid valve (TV) atrioventricular junction (AVJ) annular motion parameters in unrepaired atrial septal defect (ASD) and repaired Tetralogy of Fallot (TOF) by cardiac magnetic resonance (CMR) imaging. We retrospectively reviewed CMR studies performed between November 2007 and November 2013 in patients 16-45 years of age with unrepaired ASD (with or without partial anomalous pulmonary venous return) and with repaired TOF, who had previous infundibulotomy, but have not undergone pulmonary valve replacement. Longitudinal motion of lateral TV in four-chamber view cine image was tracked through the cardiac cycle with custom software. Twenty TOF patients and 12 ASD patients were included, and values were compared with 80 controls. Right ventricular end-diastolic volume index and right ventricular end-systolic volume index were similar in the ASD and TOF groups and were significantly higher in both groups than in controls. Maximum displacement of the TV in systole, velocity at half-maximal displacement during systole, and velocity at half-maximal displacement during early diastole were all significantly lower in the TOF group than the ASD group [1.39 ± 0.47 vs. 2.21 ± 0.46 (cm, p < 0.01), 5.9 ± 2.1 vs. 10.1 ± 2.3 (cm/s, p < 0.01), and 7.7 ± 2.6 vs. 10.9 ± 3.1 (cm/s, p < 0.05)]. TOF patients have diminished early diastolic TV AVJ velocity compared to patients with an unrepaired ASD, despite similar RV volumes. This observation could suggest diastolic dysfunction or cardiac mechanics unique to the postoperative, volume-overloaded right ventricle in patients with repaired TOF.

    View details for DOI 10.1007/s00246-015-1160-2

    View details for Web of Science ID 000357683800026

    View details for PubMedID 25835201

  • Clinical and hemodynamic outcomes up to 7 years after transcatheter pulmonary valve replacement in the US melody valve investigational device exemption trial. Circulation Cheatham, J. P., Hellenbrand, W. E., Zahn, E. M., Jones, T. K., Berman, D. P., Vincent, J. A., McElhinney, D. B. 2015; 131 (22): 1960-1970

    Abstract

    Studies of transcatheter pulmonary valve (TPV) replacement with the Melody valve have demonstrated good short-term outcomes, but there are no published long-term follow-up data.The US Investigational Device Exemption trial prospectively enrolled 171 pediatric and adult patients (median age, 19 years) with right ventricular outflow tract conduit obstruction or regurgitation. The 148 patients who received and were discharged with a TPV were followed up annually according to a standardized protocol. During a median follow-up of 4.5 years (range, 0.4-7 years), 32 patients underwent right ventricular outflow tract reintervention for obstruction (n=27, with stent fracture in 22), endocarditis (n=3, 2 with stenosis and 1 with pulmonary regurgitation), or right ventricular dysfunction (n=2). Eleven patients had the TPV explanted as an initial or second reintervention. Five-year freedom from reintervention and explantation was 76±4% and 92±3%, respectively. A conduit prestent and lower discharge right ventricular outflow tract gradient were associated with longer freedom from reintervention. In the 113 patients who were alive and reintervention free, the follow-up gradient (median, 4.5 years after implantation) was unchanged from early post-TPV replacement, and all but 1 patient had mild or less pulmonary regurgitation. Almost all patients were in New York Heart Association class I or II. More severely impaired baseline spirometry was associated with a lower likelihood of improvement in exercise function after TPV replacement.TPV replacement with the Melody valve provided good hemodynamic and clinical outcomes up to 7 years after implantation. Primary valve failure was rare. The main cause of TPV dysfunction was stenosis related to stent fracture, which was uncommon once prestenting became more widely adopted.URL: http://www.clinicaltrials.gov. Unique identifier: NCT00740870.

    View details for DOI 10.1161/CIRCULATIONAHA.114.013588

    View details for PubMedID 25944758

  • Intermediate Outcomes in the Prospective, Multicenter Coarctation of the Aorta Stent Trial (COAST) CIRCULATION Meadows, J., Minahan, M., McElhinney, D. B., McEnaney, K., Ringel, R. 2015; 131 (19): 1656-1664

    Abstract

    The Coarctation of the Aorta Stent Trial (COAST) was designed to assess the safety and efficacy of the Cheatham Platinum stent when used in children and adults with native or recurrent coarctation. Acute outcomes have been reported. We report here follow-up to 2 years.A total of 105 patients underwent attempted implantation, with 104 successes. There were no procedural deaths, serious adverse events, or surgical intervention. All patients experienced immediate reduction in upper- to lower-extremity blood pressure difference with sustained improvement to 2 years. Rates of hypertension and medication use decreased from baseline to 12 months and remained largely unchanged at 2 years. Six aortic aneurysms have been identified: 5 were successfully treated with covered stent placement, and 1 resolved without intervention. Stent fractures were noted in 2 patients at 1 year and 11 patients at 2 years, with evidence of fracture progression. To date, only larger stent diameter was associated with stent fracture. Twelve additional fractures have occurred after 2 years. No fracture has resulted in loss of stent integrity, stent embolization, aortic wall injury, or reobstruction. Nine reinterventions occurred in the first 2 years for stent redilation and address of aneurysms, and 10 additional reinterventions occurred after 2 years.The Cheatham Platinum stent is safe and associated with persistent relief of aortic obstruction. Stent fracture and progression of fracture occur but have not resulted in clinically important sequelae. Reintervention is common and related to early and late aortic wall injury and need for re-expansion of small-diameter stents.URL: http://www.clinicaltrials.gov. Unique identifier: NCT00552812.

    View details for DOI 10.1161/CIRCULATIONAHA.114.013937

    View details for Web of Science ID 000354213000011

    View details for PubMedID 25869198

  • Percutaneous tricuspid valve implantation: two-center experience with midterm results. Circulation. Cardiovascular interventions Eicken, A., Schubert, S., Hager, A., Hörer, J., McElhinney, D. B., Hess, J., Ewert, P., Berger, F. 2015; 8 (4)
  • Percutaneous tricuspid valve implantation: two-center experience with midterm results. Circulation. Cardiovascular interventions Eicken, A., Schubert, S., Hager, A., Hörer, J., McElhinney, D. B., Hess, J., Ewert, P., Berger, F. 2015; 8 (4)

    Abstract

    Severe tricuspid valve (TV) dysfunction may lead to surgical TV replacement with a biological valve prosthesis in patients with congenital heart disease. To expand the lifetime of this valve and reduce the number of surgeries, percutaneous TV implantation (PTVI) may be an effective alternative to repeated surgery. We report on our 2-center experience with PTVI.Between 2008 and 2014, 17 percutaneous valves were implanted in 16 patients with TV bioprosthesis dysfunction (9 females) from 2 centers. Median age and weight were 31.3 years (5-77.2) and 65.2 kg (17.7-107); 14 patients had congenital heart disease (univentricular heart with a right atrial to right ventricle bioprosthesis in 3, Ebstein's anomaly of the TV in 5, and other in 6), and 2 had acquired TV dysfunction. All procedures were successful (Melody n=7, Sapien 26 mm valve n=4, Sapien XT 29 mm valve n=6). One valve showed early dysfunction. It was replaced surgically and shortly after that a repeated PTVI was performed. The median duration of follow-up was 2.1 years (3 days to 6.3 years). The percutaneous valve was performing well in 15 of 16 patients.PTVI was safe and effectively improved TV function in all but 1 patient at midterm follow-up. We think that PTVI is a good alternative to repeated surgical TV replacements and that it may reduce the total number of open heart surgeries in these patients.

    View details for DOI 10.1161/CIRCINTERVENTIONS.114.002155

    View details for PubMedID 25873731

  • Tricuspid Valve Regurgitation in Congenitally Corrected Transposition of the Great Arteries and a Left Ventricle to Pulmonary Artery Conduit ANNALS OF THORACIC SURGERY Buber, J., McElhinney, D. B., Valente, A. M., Marshall, A. C., Landzberg, M. J. 2015; 99 (4): 1348-1356

    Abstract

    The configuration of the interventricular septum can affect the function of the tricuspid valve in patients with congenitally corrected transposition of the great arteries who have a systemically functioning right ventricle. Altering septal configuration by addressing a dysfunctional conduit placed between the left ventricle (LV) and the pulmonary artery (PA) in these patients can impact septal configuration and competency of the tricuspid valve.In 38 patients with an LV to PA conduit, we evaluated relationships between conduit function, RV geometry, and tricuspid valve function, and compared these variables before and after conduit intervention.Median age at conduit implant was 4.5 years (0.5 to 36) and median total follow-up was 12 years (2 to 22). Of the 38 patients, 23 (60%) underwent conduit intervention, a median of 7.5 years after implant. In 15 of these patients (65%) the degree of tricuspid regurgitation (TR) worsened, compared with only 2 patients (15%) in the non-intervention group (p < 0.001). Worsening TR was associated with the degree of change in RV and LV ventricular diameters, change in tricuspid annulus size and tethering distance, and the degree of septal shift, as reflected by the right ventricular sphericity index (all p ≤ 0.04). In 8 of 15 patients with more severe TR at follow-up, there was also progressive RV dysfunction.Intervention for LV to PA conduit dysfunction may result in worsening TR and right ventricular function, likely due in part to altered septal shift due to changes in the interventricular pressure ratio. Management of LV to PA conduit dysfunction should take these findings into account.

    View details for DOI 10.1016/j.athoracsur.2014.11.008

    View details for Web of Science ID 000352162100045

    View details for PubMedID 25661908

  • Low rate of prenatal diagnosis among neonates with critical aortic stenosis: insight into the natural history in utero ULTRASOUND IN OBSTETRICS & GYNECOLOGY Freud, L. R., Moon-Grady, A., Escobar-Diaz, M. C., Gotteiner, N. L., Young, L. T., McElhinney, D. B., Tworetzky, W. 2015; 45 (3): 326-332

    Abstract

    To better understand the natural history and spectrum of fetal aortic stenosis (AS), we aimed to (1) determine the prenatal diagnosis rate of neonates with critical AS and a biventricular (BV) outcome, and (2) describe the findings at fetal echocardiography in patients diagnosed prenatally.A multicenter, retrospective study was performed on neonates who presented with critical AS and who were discharged with a BV outcome from 2000 to 2013. The prenatal diagnosis rate was compared with that reported for hypoplastic left heart syndrome (HLHS). We reviewed fetal echocardiographic findings in patients who were diagnosed prenatally.In only 10 (8.5%) of 117 neonates with critical AS and a BV outcome was the diagnosis made prenatally, a rate significantly lower than that for HLHS in the contemporary era (82%; P < 0.0001). Of the 10 patients diagnosed prenatally, all had developed left ventricular dysfunction by a median gestational age of 33 (range, 28-35) weeks. When present, Doppler abnormalities such as retrograde flow in the aortic arch (n = 2), monophasic mitral inflow (n = 3) and left-to-right flow across the foramen ovale (n = 8) developed late in gestation (median 33 weeks).The prenatal diagnosis rate of critical AS and a BV outcome among neonates is very low, probably owing to a relatively normal four-chamber view in mid-gestation with development of significant obstruction in the third trimester. The natural history contrasts with that of severe mid-gestation AS with evolving HLHS and suggests that the gestational timing of development of significant AS has an important impact on subsequent left-heart growth in utero.

    View details for DOI 10.1002/uog.14667

    View details for Web of Science ID 000350641200014

    View details for PubMedID 25251721

  • Comparison of Reported Outcomes With Percutaneous Versus Surgical Closure of Ruptured Sinus of Valsalva Aneurysm AMERICAN JOURNAL OF CARDIOLOGY Kuriakose, E. M., Bhatla, P., McElhinney, D. B. 2015; 115 (3): 392-398

    Abstract

    Sinus of Valsalva aneurysm is a rare cardiac malformation that stems from incomplete fusion of the aortic media and the aortic valve annulus, a weakness that may result in rupture of the sinus, large left-to-right shunt, and severe congestive heart failure. Historically, this lesion has been repaired surgically, but percutaneous closure (PC) has emerged as a therapeutic intervention over the last 20 years. We review and contrast 34 studies detailing the PC approach with 16 studies on surgical closure (SC), together comprising a total of 877 patients who were treated for ruptured sinus of Valsalva aneurysm from 1956 to 2014. Both groups had similar sites of rupture, age distribution, and clinical symptoms at presentation. Selection bias ultimately prohibits a direct comparison between the 2 groups as patients who underwent SC often had worse aortic regurgitation and more complex associated lesions, including endocarditis, bicuspid aortic valve, tunnel-type fistulous connections, larger defect size, and multiple site of rupture. In conclusion, although SC is indicated and reserved for these more complicated patients, our review of previously published reports reveals that PC in patients who are too ill to undergo bypass, with mild or no aortic regurgitation and simple associated defects (muscular ventricular septal defects, secundum atrial septal defect, small patent ductus arteriosus), can be safe, effective, and practical.

    View details for DOI 10.1016/j.amjcard.2014.11.013

    View details for Web of Science ID 000348629400019

    View details for PubMedID 25488356

  • Management of Cardiovascular Risk Factors in Adults With Congenital Heart Disease JOURNAL OF THE AMERICAN HEART ASSOCIATION Lui, G. K., Fernandes, S., McElhinney, D. B. 2014; 3 (6)
  • Pulmonary Deadspace and Postoperative Outcomes in Neonates Undergoing Stage 1 Palliation Operation for Single Ventricle Heart Disease PEDIATRIC CRITICAL CARE MEDICINE Shakti, D., McElhinney, D. B., Gauvreau, K., Yarlagadda, V. V., Laussen, P. C., Betit, P., Myrer, M. L., Thiagarajan, R. R. 2014; 15 (8): 728-734

    Abstract

    Increased pulmonary dead space fraction (VD/VT) has been associated with prolonged mechanical ventilation after surgery for congenital heart disease. The association of VD/VT with clinical outcomes in neonates undergoing stage 1 palliation for single ventricle congenital heart disease has not been reported. We describe changes in VD/VT, differences in VD/VT based on shunt type (right ventricle to pulmonary artery conduit vs modified Blalock-Taussing shunt) and association of VD/VT with postoperative outcomes in patients undergoing stage 1 palliation.Retrospective chart review for demographic, hemodynamics, outcome information, and VD/VT values were collected at 6-hour intervals during the first 48 postoperative hours in neonates undergoing stage 1 palliation. VD/VT was calculated using mixed expired CO2 (PeCO2) obtained from capnography and paired arterial blood gas CO2 values.Cardiac ICU in a tertiary care pediatric hospital.Newborns with single ventricle congenital heart disease undergoing stage 1 palliation during 2003-2004.Of the 51 patients, 31 had right ventricle to pulmonary artery and 20 had Blalock-Taussing shunt. Although VD/VT was lower in the Blalock-Taussing shunt group over all time points (p = 0.02), maximal VD/VT on day 1 (0.49 ± 0.07) and on day 2 (0.46 ± 0.08) were not different between the shunt groups. VD/VT decreased significantly over time in both shunt groups (p = 0.001 for right ventricle to pulmonary artery; p < 0.001 for Blalock-Taussing shunt). Higher maximal VD/VT during first 48 postoperative hours was independently associated with fewer ventilator (β = -26.6; p = 0.035) and hospital-free days in the first month after stage 1 palliation (β = -40.4; p = 0.002) after adjusting for potential confounders in a multivariable linear regression model.Increased pulmonary dead space exists early after stage 1 palliation operation for single ventricle congenital heart disease. Higher VD/VT during the first 48 postoperative hours was associated with longer duration of ventilation and hospital LOS and may be a useful marker of postoperative outcomes in this population.

    View details for DOI 10.1097/PCC.0000000000000226

    View details for Web of Science ID 000343049200010

    View details for PubMedID 25072474

  • Durability of large diameter right ventricular outflow tract conduits in adults with congenital heart disease INTERNATIONAL JOURNAL OF CARDIOLOGY Buber, J., Assenza, G. E., Huang, A., Valente, A. M., Emani, S. M., Gauvreau, K., Marshal, A. C., McElhinney, D. B., Landzberg, M. J. 2014; 175 (3): 455-463

    Abstract

    Subpulmonary ventricular outflow conduits are utilized routinely to repair complex congenital cardiac abnormalities, but are limited by the inevitable degeneration and need for reintervention. Data on conduit durability and propensity to dysfunction in the adult population are limited.The study included 288 consecutive patients ≥18 years of age who were evaluated between 1991 and 2010 after placement of a ≥18 mm conduit. Freedom from hemodynamic conduit dysfunction served as our primary outcome. Freedom from reintervention, overall mortality and heart transplantation were also evaluated.Median age at conduit implant was 19 years and median follow-up duration was 13 years. Probabilities of survival without conduit dysfunction and reintervention at 5, 10 and 15 years were 87%, 63%, and 49%, and 95%, 81%, and 56%, respectively. Smaller conduit diameter (18-20mm) was associated with lower probability of survival without dysfunction in the entire study cohort, with prominent effects in patients in both the lowest and the highest age quartiles. Other parameters with similar associations were higher BMI, native anatomy of tetralogy of Fallot or truncus arteriosus, and active smoking.Adult congenital heart disease patients with conduit diameter ≥18 mm had an approximately 50% chance of developing hemodynamic conduit dysfunction and undergoing conduit reintervention by 15 years of post-implant, and a 30% likelihood of undergoing conduit reoperation in the same time frame. The importance of these data is underscored by the increasing number of adults with congenital heart diseases seeking care and the recent advances in transcatheter valve replacement for dysfunctional conduits.

    View details for DOI 10.1016/j.ijcard.2014.06.023

    View details for Web of Science ID 000340251600015

    View details for PubMedID 25002319

  • Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome Postnatal Outcomes of the First 100 Patients CIRCULATION Freud, L. R., McElhinney, D. B., Marshall, A. C., Marx, G. R., Friedman, K. G., del Nido, P. J., Emani, S. M., Lafranchi, T., Silva, V., Wilkins-Haug, L. E., Benson, C. B., Lock, J. E., Tworetzky, W. 2014; 130 (8): 638-?

    Abstract

    Fetal aortic valvuloplasty can be performed for severe midgestation aortic stenosis in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after fetal aortic valvuloplasty. The postnatal outcomes and survival of the BV patients, in comparison with those managed as HLHS, have not been reported.We included 100 patients who underwent fetal aortic valvuloplasty for severe midgestation aortic stenosis with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and left ventricular volume, were significantly larger in the BV group at the time of birth (P<0.01). After a median follow-up of 5.4 years, freedom from cardiac death among all BV patients was 96±4% at 5 years and 84±12% at 10 years, which was better than HLHS patients (log-rank P=0.04). There was no cardiac mortality in patients with a BV circulation from birth. All but 1 of the BV patients required postnatal intervention; 42% underwent aortic or mitral valve replacement. On the most recent echocardiogram, the median left ventricular end-diastolic volume z score was +1.7 (range, -1.3 to +8.2), and 80% had normal ejection fraction.Short- and intermediate-term survival among patients who underwent fetal aortic valvuloplasty and achieved a BV circulation postnatally is encouraging. However, morbidity still exists, and ongoing assessment is warranted.

    View details for DOI 10.1161/CIRCULATIONAHA.114.009032

    View details for Web of Science ID 000341300100011

    View details for PubMedID 25052401

  • Technical Challenges of Atrial Septal Stent Placement in Fetuses with Hypoplastic Left Heart Syndrome and Intact Atrial Septum CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Kalish, B. T., Tworetzky, W., Benson, C. B., Wilkins-Haug, L., Mizrahi-Arnaud, A., McElhinney, D. B., Lock, J. E., Marshall, A. C. 2014; 84 (1): 77-85

    Abstract

    The objective of this study was to describe our single-institution experience with prenatal atrial septal stent placement for fetuses with hypoplastic left heart syndrome and an intact atrial septum (HLHS/IAS).Infants born with HLHS/IAS are at high risk for neonatal death, despite maximal postnatal therapy. Prenatal atrial septoplasty by static balloon dilation has been effective in decompressing the left atrium (LA) in utero, but several factors have limited the size of septal defects. We attempted to overcome the limitations of balloon septoplasty using transcatheter atrial septal stents.All records from our institution of fetuses with HLHS/IAS that underwent prenatal atrial septal stent placement were reviewed, including operative notes and echocardiograms.Nine fetuses between 24 and 31 weeks gestation with HLHS/IAS underwent attempted fetal atrial septal stent placement. A stent was deployed across the atrial septum in five fetuses, with four fetuses demonstrating flow across the stent at the time of intervention. In four cases, stent placement failed due to malposition or embolization, but in three of the four cases, atrial balloon septoplasty at the same in-utero procedure successfully and acutely decompressed the LA. There were no maternal complications. There was one fetal demise. The remaining eight fetuses survived to delivery, but four died in the neonatal period (two of which had been stented).Ultrasound-guided atrial septal stent placement is feasible in some fetuses with HLHS/IAS. Visualization of the septum and catheter tip is critical to technical success. Additional experience is necessary to determine the clinical impact of this intervention. © 2013 Wiley Periodicals, Inc.

    View details for DOI 10.1002/ccd.25098

    View details for Web of Science ID 000337970100018

    View details for PubMedID 23804575

  • Use and Performance of the Melody Transcatheter Pulmonary Valve in Native and Postsurgical, Nonconduit Right Ventricular Outflow Tracts CIRCULATION-CARDIOVASCULAR INTERVENTIONS Meadows, J. J., Moore, P. M., Berman, D. P., Cheatham, J. P., Cheatham, S. L., Porras, D., Gillespie, M. J., Rome, J. J., Zahn, E. M., McElhinney, D. B. 2014; 7 (3): 374-380

    Abstract

    Melody Transcatheter Pulmonary Valve (TPV) replacement therapy represents an important advance in congenital cardiovascular interventions. The off-label extension of the Melody TPV to patients with nonconduit outflow tracts (right ventricular outflow tract [RVOT]) has the potential to vastly expand the population of patients eligible to benefit from nonsurgical restoration of RVOT function. However, knowledge on the performance of the Melody TPV in this setting is limited.This is a multicenter, retrospective review of the Melody TPV when placed in nonconduit RVOTs, in which at least a portion of the circumference was composed of native tissue. Five centers contributed data on 31 patients. The median age at implantation was 24 years (range, 7-66). At a median follow-up of 15 months, all patients were alive. No patient had greater than mild TPV insufficiency, and the median maximum instantaneous gradients across the RVOT was 23 mm Hg. Stent fracture occurred in 32%. Eight patients developed more than mild TPV obstruction, of whom 6 were associated with identified stent fracture. Three patients developed blood stream infections. There were 5 reinterventions in 3 patients, including 3 repeat TPV implantations and 2 TPV explantations.Melody TPV implantation is feasible in selected patients with RVOTs comprised solely or predominantly native tissue and has the potential to expand the population of patients eligible to benefit from nonsurgical restoration of RVOT function. In early follow-up, valve competency seems preserved. The dominant mechanism of valve dysfunction seems to be related to stent fracture with recurrent obstruction. Additional data are necessary to better understand how to safely expand TPV therapy to this population.

    View details for DOI 10.1161/CIRCINTERVENTIONS.114.001225

    View details for Web of Science ID 000337746700013

    View details for PubMedID 24867892

  • Feasibility and Short-Term Outcomes of Percutaneous Transcatheter Pulmonary Valve Replacement in Small (< 30 kg) Children With Dysfunctional Right Ventricular Outflow Tract Conduits CIRCULATION-CARDIOVASCULAR INTERVENTIONS Berman, D. P., McElhinney, D. B., Vincent, J. A., Hellenbrand, W. E., Zahn, E. M. 2014; 7 (2): 142-148

    Abstract

    In 2010, the Melody transcatheter pulmonary valve (TPV) received Food and Drug Administration approval for treatment of dysfunctional right ventricular outflow tract conduits in patients ≥30 kg. Limited data are available regarding use of this device in smaller patients.We evaluated technical and short-term clinical outcomes of 25 patients <30 kg (10 patients <20 kg) who underwent TPV replacement for treatment of conduit dysfunction at 3 centers. Median age and weight were 8.0 years (3.4-14.4) and 21.4 kg (13.8-29.0). The median conduit diameter at the time of surgical implant was 17 mm (12-23). Two patients did not undergo TPV implant (risk of coronary compression in 1; inability to advance the delivery sheath beyond the common femoral vein in 1). After successful TPV implant, the peak conduit gradient fell from 29±16 to 9±6 mm Hg (P<0.001), and all but 2 patients had no/trivial regurgitation (down from moderate or severe preimplant in 20). TPV implant was via the femoral vein in 17 patients, the right internal jugular vein in 4, and the left subclavian vein in 2 patients. At a median follow-up of 16 months, 1 patient underwent conduit replacement for recurrent conduit stenosis, 2 developed stent fracture requiring a second TPV, and 2 developed bacterial endocarditis treated with antibiotics, 1 of whom then underwent conduit replacement. The average Melody valve mean Doppler gradient and conduit regurgitation were unchanged from early postimplant.Percutaneous TPV replacement can be performed in small children with good procedural and early hemodynamic results in the majority of patients.

    View details for DOI 10.1161/CIRCINTERVENTIONS.113.000881

    View details for Web of Science ID 000337746200004

    View details for PubMedID 24569596

  • Angioplasty of Obstructed Homograft Conduits in the Right Ventricular Outflow Tract With Ultra-Noncompliant Balloons Assessment of Therapeutic Efficacy and Conduit Tears CELL BIOLOGY INTERNATIONAL Hainstock, M. R., Marshall, A. C., Lock, J. E., McElhinney, D. B. 2014; 38 (1): 671-679
  • Angioplasty of Obstructed Homograft Conduits in the Right Ventricular Outflow Tract With Ultra-Noncompliant Balloons Assessment of Therapeutic Efficacy and Conduit Tears CIRCULATION-CARDIOVASCULAR INTERVENTIONS Hainstock, M. R., Marshall, A. C., Lock, J. E., McElhinney, D. B. 2013; 6 (6): 671-679

    Abstract

    Angioplasty and stent placement in right ventricle-to-pulmonary artery (RV-PA) conduits have been shown to prolong the functional lifespan of a conduit. Safety and efficacy of angioplasty of obstructed RV-PA homografts using ultra-noncompliant (UNC) or ultrahigh-pressure balloons are unknown.From 2004 to 2012, 70 patients underwent 76 procedures for angioplasty of RV-PA homografts with UNC Atlas balloons. The UNC group was compared with a partially contemporaneous control cohort of 81 patients who underwent 84 angioplasty procedures with conventional balloons. Acute hemodynamic changes after angioplasty of homografts with UNC balloons included significantly reduced RV:Ao pressure ratio (P=0.02) and right ventricular outflow tract gradients (P≤0.001). Balloon waist resolution was more frequently achieved with UNC balloons (P=0.04), and balloon rupture occurred less often (P<0.001). Conduit tears of any severity occurred in 22% of patients overall and were more common in the UNC group (P=0.001). Patients with any conduit tear had significantly greater reduction in their RV:Ao pressure ratio (P<0.001) and right ventricular outflow tract gradient (P=0.004) than those with no tear. There were 4 unconfined tears, all in the UNC group, with no acute decompensations or deaths and only 1 patient who required surgical management.RV-PA conduit tears are common in patients undergoing angioplasty, but clinically important tears, which only occurred during UNC angioplasty in this series, were uncommon. UNC balloons can be used to good effect with significant reduction in right ventricular outflow tract gradient and the RV:Ao ratio when compared with conventional balloons.

    View details for DOI 10.1161/CIRCINTERVENTIONS.112.000073

    View details for Web of Science ID 000328511300014

    View details for PubMedID 24254707

  • Heterogeneity of Regional Function and Relation to Ventricular Morphology in Patients With Fontan Circulation AMERICAN JOURNAL OF CARDIOLOGY Lunze, F. I., Lunze, K., McElhinney, D. B., Colan, S. D., Gauvreau, K., Lange, P. E., Schmitt, B., Berger, F. 2013; 112 (8): 1207-1213

    Abstract

    The relation between underlying ventricular morphology and regional function in patients with Fontan circulation remains unclear. The aim of this study was to compare regional function and its heterogeneity in patients with tricuspid atresia (TA), biventricular apex-forming morphology (BiV), and controls. Nineteen patients (median age 12 years) with Fontan circulation who presented consecutively were prospectively enrolled and compared with age- and heart rate-matched controls. Most patients were in New York Heart Association class I (63%). Longitudinal systolic strain (S), systolic strain rate (SRsys), and early diastolic strain rate (SRdia) peaks were obtained from 6 ventricular segments, and a coefficient of variation by segment was calculated as a measure of regional heterogeneity. Systolic S, SRsys and SRdia peaks were decreased at the right and left lateral walls in both patient groups compared with controls (p ≤0.001 for all). Patients with TA had higher systolic S and SRsys in the middle of the right lateral wall than those with BiV morphology (p = 0.009 and p = 0.001, respectively). The mean coefficients of variation assessed by S and SRsys were similar in controls and patients with TA but lower in those with BiV than in controls and patients with TA (p <0.001 and p = 0.01, respectively). The mean coefficient of variation assessed by SRdia was greater only in patients with BiV than in controls (p = 0.001). In conclusion, patients with Fontan circulation have more heterogeneous systolic and early diastolic regional function than healthy control subjects, and patients with TA have better systolic regional function in the middle of the right lateral wall and less systolic heterogeneity than patients with BiV morphology.

    View details for DOI 10.1016/j.amjcard.2013.06.016

    View details for Web of Science ID 000325833700024

    View details for PubMedID 23927787

  • Fracture of Cardiovascular Stents in Patients With Congenital Heart Disease Theoretical and Empirical Considerations CIRCULATION-CARDIOVASCULAR INTERVENTIONS McElhinney, D. B., Marshall, A. C., Schievano, S. 2013; 6 (5): 575-585
  • Risk of Coronary Artery Compression Among Patients Referred for Transcatheter Pulmonary Valve Implantation A Multicenter Experience CIRCULATION-CARDIOVASCULAR INTERVENTIONS Morray, B. H., McElhinney, D. B., Cheatham, J. P., Zahn, E. M., Berman, D. P., Sullivan, P. M., Lock, J. E., Jones, T. K. 2013; 6 (5): 535-542

    Abstract

    The Melody transcatheter pulmonary valve (TPV) was approved for implantation in obstructed right ventricular outflow tract conduits in 2010 after a multicenter trial demonstrating improvements in conduit obstruction, regurgitation, and right ventricular pressure. A recognized risk and contraindication to TPV implantation is the demonstration of coronary artery (CA) compression during balloon angioplasty or stent placement in the overlying conduit. This study is the first to characterize the risk of CA compression in this population.From 2007 to 2012, 404 patients underwent 407 catheterizations for potential TPV implantation (median age, 18 years) at 4 centers. Three hundred forty-three patients (85%) underwent valve implantation. Twenty-one patients (5%) had evidence of CA compression with simultaneous right ventricular outflow tract angioplasty and CA angiography. Sixty-eight patients (17%) had abnormal CA anatomy. Fifteen of 21 (71%) patients with CA compression had abnormal CA anatomy. Eight patients with tetralogy of Fallot and 7 patients with transposition of the great arteries demonstrated compression. Of the 34 patients with tetralogy of Fallot and abnormal CA, 7 (21%) demonstrated CA compression.CA compression following TPV implantation can be catastrophic. CA compression was observed in 5% of patients during test balloon angioplasty. No patients in this study developed clinically apparent CA compression after TPV implantation. CA compression was significantly associated with the presence of abnormal CA anatomy, especially in patients with tetralogy of Fallot or transposition of the great arteries. Preimplantation coronary angiography with simultaneous test angioplasty is an important step to evaluate for the presence of CA compression during TPV implantation.

    View details for DOI 10.1161/CIRCINTERVENTIONS.113.000202

    View details for Web of Science ID 000329923100011

    View details for PubMedID 24065444

  • Relationship between procedural adverse events associated with cardiac catheterization for congenital heart disease and operator factors: Results of a multi-institutional registry (C3PO) CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Holzer, R. J., Gauvreau, K., Kreutzer, J., Moore, J. W., McElhinney, D. B., Bergersen, L. 2013; 82 (3): 463-473

    Abstract

    Data examining the effect of operator years in practice and volume on adverse events (AE) after cardiac catheterization in patients with congenital heart disease is limited.Data were prospectively collected using a multi-center registry (C3PO). 10,885 catheterizations performed between 02/07 and 06/10 at eight institutions were included. AE rates were risk-adjusted for hemodynamic vulnerability, procedure type risk group, and age and compared between operators with different years in practice (YIP) and volume. AE occurred in 13% of procedures. Operators with less than five YIP had higher adjusted odds of any AE (OR 1.42, 95% CI 1.14-1.77) or a high severity AE (OR 1.35, 95% CI 1.04-1.75), when compared with operators with 5 to less than 25 YIP (5 < 25), while operators with ≥25 YIP had higher odds of a high severity (but not any) AE (OR 1.39, 95% CI 1.08-1.80). Operators with <5 YIP had a higher percentage of preventable AE (out of all AE, 16% vs. 8%, P < 0.001) as well as higher odds of vascular or cardiac trauma (OR 1.81, 95% CI 1.11-2.97), or technical AE (OR 1.98, 95% CI 1.31-2.99) when compared with operators with 5 < 25 YIP. There was no consistent relationship between operator volume, and incidence of AE.Operators with less than 5 years in practice have higher risk-adjusted AE rates. While an important consideration in guiding and mentoring operators with fewer years in practice, it is important to emphasize that reporting adverse events does not take into account procedural efficacy.

    View details for DOI 10.1002/ccd.24866

    View details for Web of Science ID 000323454100047

    View details for PubMedID 23413194

  • Outcomes After Primary Transcatheter Therapy in Infants and Young Children With Severe Bilateral Peripheral Pulmonary Artery Stenosis CIRCULATION-CARDIOVASCULAR INTERVENTIONS Cunningham, J. W., McElhinney, D. B., Gauvreau, K., Bergersen, L., Lacro, R. V., Marshall, A. C., Smoot, L., Lock, J. E. 2013; 6 (4): 460-467

    Abstract

    Angioplasty and stent implantation have become accepted therapies for isolated peripheral pulmonary stenosis, and have been shown to increase vessel diameter and reduce right ventricular (RV) pressure acutely in patients with pulmonary artery (PA) stenosis. The purpose of this study was to assess long-term outcomes after primary transcatheter therapy for peripheral pulmonary stenosis.We studied 69 patients who underwent primary transcatheter intervention for severe isolated peripheral pulmonary stenosis at ≤ 5 years of age. Genetic/syndromic diagnoses included Williams syndrome (n=23), non-Williams familial arteriopathy (n=12), and Alagille syndrome (n=3). At the initial PA intervention, median RV:aortic pressure ratio decreased from 1.00 to 0.88 (median decrease, 0.18; P<0.001). Patients with a higher preintervention RV:aortic pressure ratio had a greater reduction (P<0.001). During follow-up (median, 8.5 years), 10 patients died, 5 from complications of PA catheterization (all before 1998). Thirteen patients underwent surgical PA intervention, most within 1 year and along with repair of supravalvar aortic stenosis. Freedom from any PA reintervention was 38 ± 6% at 1 year and 22 ± 6% at 5 years. The median RV:aortic pressure ratio decreased from 1.0 at baseline to 0.53 at the most recent catheterization (P<0.001), and 82% of patients with available clinical follow-up were asymptomatic.Transcatheter therapy for infants with severe peripheral pulmonary stenosis has become safer, regardless of genetic condition. Coupled with reintervention and surgical relief in selected cases, RV:aortic pressure ratios decrease substantially and most patients are asymptomatic at late follow-up.

    View details for DOI 10.1161/CIRCINTERVENTIONS.112.000061

    View details for Web of Science ID 000323323300021

    View details for PubMedID 23941859

  • Echocardiographic predictors of left ventricular dysfunction after aortic valve surgery in children with chronic aortic regurgitation. Congenital heart disease Selamet Tierney, E. S., Gal, D., Gauvreau, K., Zhou, J., Soluk, Y., McElhinney, D. B., Colan, S. D., Geva, T. 2013; 8 (4): 308-315

    Abstract

    OBJECTIVE: Postoperative left ventricular dysfunction is associated with poor prognosis in adults with severe chronic aortic regurgitation and published practice guidelines aim to minimize this risk. However, only limited information exists in pediatrics. The goal of this study was to define preoperative risk factors for postoperative left ventricular dysfunction in children with chronic aortic regurgitation. METHODS: Patients fulfilling the following criteria were included in this study: (1) age at preoperative echocardiogram ≤18 years; (2) ≥moderate aortic regurgitation; (3) ≤mild aortic valve stenosis; (4) no additional valve disease/shunt; (5) underwent aortic valve surgery for aortic regurgitation; and (6) available preoperative and ≥6-month postoperative echocardiograms with adequate information. Primary outcome was postoperative left ventricular dysfunction defined as ejection fraction z-score < -2. RESULTS: Median ages at diagnosis and surgery of the 53 eligible patients were 6.9 (0.04-17.2) and 13 years (1.2-22.4), respectively. Compared with patients whose postoperative left ventricular ejection fraction was normal, those with left ventricular ejection fraction z-score < -2 (n = 10) had significantly higher preoperative left ventricular end-diastolic and systolic volumes and dimensions and lower indices of systolic function. Preoperative left ventricular ejection fraction z-score < -1 was the most sensitive (89%; confidence interval [CI] 52, 100) but least specific (58%; CI 41, 73), whereas left ventricular end-systolic diameter z-score ≥ 5 was the most specific (95%; CI 84, 99) but least sensitive (60%; CI 26, 88) outcome identifier. A combination of shortening fraction z-score < -1 or end-systolic diameter z-score ≥ 5 best identified postoperative left ventricular dysfunction with an area of 0.819 under the receiver-operator characteristic curve. CONCLUSION: Lower indices of left ventricular systolic function and severity of dilation identify children at risk for postoperative left ventricular dysfunction after aortic valve surgery. These identifiers are similar to predictors defined in adult patients albeit with different threshold values.

    View details for DOI 10.1111/chd.12009

    View details for PubMedID 23075071

  • Wire-related Pulmonary Artery Injury during Pediatric and Adult Congenital Interventional Cardiac Catheterization CONGENITAL HEART DISEASE Esch, J. J., Bergersen, L., McElhinney, D. B., Porras, D., Lock, J. E., Marshall, A. C. 2013; 8 (4): 296-301

    Abstract

    Guidewires used in pediatric catheterization are typically floppy, soft, or J-tipped, and are generally assumed to be atraumatic. A recent sentinel case suggested that such wires may cause clinically significant pulmonary artery (PA) injury. We sought to determine the incidence of wire-related PA injury as a cause of "idiopathic" airway bleeding (endobronchial blood) during interventional cardiac catheterization in patients with congenital heart disease.The Children's Hospital Boston database of cardiac catheterizations was reviewed for adverse events (AEs) indicating possible PA injury occurring between September 2006 and August 2011. Procedure notes were reviewed, and when the clinical scenario was suggestive of wire injury or was not clear, relevant angiograms were reviewed.One thousand forty-seven cases involving PA dilation were performed in the period of interest. Five cases of probable wire injury were identified, suggesting an incidence of approximately 0.5 per 100 cases. Of these five cases, trauma was judged due to floppy-tipped wires in two, soft-tipped wires in two, and a J-tipped wire in one. In three cases, the distal wire was looped such that the leading segment was stiffer than the wire tip. Clinical manifestations of wire injury comprised contrast within the airway, vessel aneurysm/tear, obstructive intimal flap, blood from the endotracheal tube, hemothorax, and wedge defect on lung scan. These injuries were relatively benign and did not result in instability or prolonged bleeding.Wire injury to the PAs is relatively uncommon, although possible with even floppy-tipped wires. The mechanism and implications of such injuries are markedly different than balloon-mediated vascular tears.

    View details for DOI 10.1111/chd.12015

    View details for Web of Science ID 000326501000012

    View details for PubMedID 23075214

  • Echocardiographic Predictors of Left Ventricular Dysfunction after Aortic Valve Surgery in Children with Chronic Aortic Regurgitation CONGENITAL HEART DISEASE Tierney, E. S., Gal, D., Gauvreau, K., Zhou, J., Soluk, Y., McElhinney, D. B., Colan, S. D., Geva, T. 2013; 8 (4): 308-315

    Abstract

    OBJECTIVE: Postoperative left ventricular dysfunction is associated with poor prognosis in adults with severe chronic aortic regurgitation and published practice guidelines aim to minimize this risk. However, only limited information exists in pediatrics. The goal of this study was to define preoperative risk factors for postoperative left ventricular dysfunction in children with chronic aortic regurgitation. METHODS: Patients fulfilling the following criteria were included in this study: (1) age at preoperative echocardiogram ≤18 years; (2) ≥moderate aortic regurgitation; (3) ≤mild aortic valve stenosis; (4) no additional valve disease/shunt; (5) underwent aortic valve surgery for aortic regurgitation; and (6) available preoperative and ≥6-month postoperative echocardiograms with adequate information. Primary outcome was postoperative left ventricular dysfunction defined as ejection fraction z-score < -2. RESULTS: Median ages at diagnosis and surgery of the 53 eligible patients were 6.9 (0.04-17.2) and 13 years (1.2-22.4), respectively. Compared with patients whose postoperative left ventricular ejection fraction was normal, those with left ventricular ejection fraction z-score < -2 (n = 10) had significantly higher preoperative left ventricular end-diastolic and systolic volumes and dimensions and lower indices of systolic function. Preoperative left ventricular ejection fraction z-score < -1 was the most sensitive (89%; confidence interval [CI] 52, 100) but least specific (58%; CI 41, 73), whereas left ventricular end-systolic diameter z-score ≥ 5 was the most specific (95%; CI 84, 99) but least sensitive (60%; CI 26, 88) outcome identifier. A combination of shortening fraction z-score < -1 or end-systolic diameter z-score ≥ 5 best identified postoperative left ventricular dysfunction with an area of 0.819 under the receiver-operator characteristic curve. CONCLUSION: Lower indices of left ventricular systolic function and severity of dilation identify children at risk for postoperative left ventricular dysfunction after aortic valve surgery. These identifiers are similar to predictors defined in adult patients albeit with different threshold values.

    View details for DOI 10.1111/chd.12009

    View details for Web of Science ID 000326501000014

  • Infective Endocarditis After Transcatheter Pulmonary Valve Replacement Using the Melody Valve: Combined Results of 3 Prospective North American and European Studies CIRCULATION-CARDIOVASCULAR INTERVENTIONS McElhinney, D. B., Benson, L. N., Eicken, A., Kreutzer, J., Padera, R. F., Zahn, E. M. 2013; 6 (3): 292-300

    Abstract

    Transcatheter (percutaneous) pulmonary valve (TPV) replacement has emerged as a viable therapy for right ventricular outflow tract conduit dysfunction. Little is known about the incidence, clinical course, and outcome of infective endocarditis (IE) after TPV implant. We reviewed combined data from 3 ongoing prospective multicenter trials to evaluate the experience with IE among patients undergoing TPV replacement using the Melody valve.Any clinical episode reported by investigators as IE with documented positive blood cultures and fever, regardless of TPV involvement, was considered IE. Cases were classified as TPV-related if there was evidence of vegetations on or new dysfunction of the TPV. The 3 trials included 311 patients followed for 687.1 patient-years (median, 2.5 years). Sixteen patients were diagnosed with IE 50 days to 4.7 years after TPV implant (median, 1.3 years), including 6 who met criteria for TPV-related IE: 3 with vegetations, 2 with TPV dysfunction, and 1 with both. The annualized rate of a first episode of IE was 2.4% per patient-year and of TPV-related IE was 0.88% per patient-year. Freedom from TPV-related IE was 97±1% 4 years after implant. All patients were treated with intravenous antibiotics, 4 had the valve explanted, and 2 received a second TPV. There was 1 sepsis-related death, 1 patient died of sudden hemoptysis, and 2 patients developed recurrent IE.Bacterial endocarditis has occurred in all 3 prospective multicenter studies of the Melody valve in North America and Europe. Most cases did not involve the TPV and responded to antibiotics. More data are necessary to understand risk factors in this population.

    View details for DOI 10.1161/CIRCINTERVENTIONS.112.000087

    View details for Web of Science ID 000320759800020

    View details for PubMedID 23735475

  • Transcatheter Closure of Post-myocardial Infarction Ventricular Septal Rupture CIRCULATION-CARDIOVASCULAR INTERVENTIONS Assenza, G. E., McElhinney, D. B., Valente, A. M., Pearson, D. D., Volpe, M., Martucci, G., Landzberg, M. J., Lock, J. E. 2013; 6 (1): 59-?

    Abstract

    Ventricular septal rupture (VSR) after acute myocardial infarction (AMI) is a potentially lethal mechanical complication of acute coronary syndromes. Given high surgical mortality, transcatheter closure has emerged as a potential strategy in selected cases. We report our single-center experience with double-umbrella device percutaneous closure of post-AMI VSR.In this single-center, retrospective, cohort study, patients who underwent transcatheter closure of post-AMI VSR between 1988 and 2008 at Boston Children's Hospital were included. Data were analysed according to whether the patients underwent direct percutaneous VSR closure or closure of a residual VSR after a previous surgical approach. Primary outcome was mortality rate at 30 days. Clinical predictors of primary outcome were investigated using univariate logistic regression. Thirty patients were included in the study (mean age, 67±8 years). A total of 40 closure devices were implanted. Major periprocedural complications occurred in 4 (13%) patients. Cardiogenic shock, increasing pulmonary/systemic flow ratio, and the use of the new generation (6-arm) STARFlex device all were associated with higher risk of mortality. The Model for End-Stage Liver Disease Excluding international normalized ratio (MELD-XI) score at the time of VSR closure seemed to be most strongly associated with death (odds ratio, 1.6; confidence interval, 1.1-2.2; P<0.001).Transcatheter closure of post-AMI VSR using CardioSEAL or STARFlex devices is feasible and effective. The MELD-XI score, a marker of multiorgan dysfunction, is a promising risk stratifier in this population of patients. Early closure of post-AMI VSR is advisable before establishment of multiorgan failure.

    View details for DOI 10.1161/CIRCINTERVENTIONS.112.972711

    View details for Web of Science ID 000330360900016

    View details for PubMedID 23339839

  • Left Ventricular Diastolic Function in Children and Young Adults With Congenital Aortic Valve Disease AMERICAN JOURNAL OF CARDIOLOGY Friedman, K. G., McElhinney, D. B., Rhodes, J., Powell, A. J., Colan, S. D., Lock, J. E., Brown, D. W. 2013; 111 (2): 243-249

    Abstract

    Young patients with congenital aortic valve disease are at risk of left ventricular (LV) diastolic dysfunction (DD). We evaluated LV remodeling and the prevalence of, and risk factors for, DD in patients with aortic stenosis (AS), pure aortic regurgitation (AR), and AS+AR. Patients aged 8 to 39 years with congenital AS (n = 103), AR (n = 36), or AS+AR (n = 107) were identified. Cross-sectional assessment of the LV remodeling pattern and diastolic function was performed. A diastolic function score (DFS; range 0 to 4) was assigned to each patient, with 1 point for an abnormal value in each of 4 categories: mitral inflow (E/A and E-wave deceleration time), tissue Doppler E', E/E', and left atrial volume. Patients with a DFS of ≥2 were compared to those with a DFS <2. Concentric hypertrophy was the most common remodeling pattern in those with AS (51%), mixed/physiologic hypertrophy in those with AS+AR (48%) and eccentric hypertrophy in those with AR (49%) predominated. In the entire cohort, 91 patients (37%) had a DFS of ≥2. Patients with AS or AS+AR had greater DFS than those with pure AR (p <0.001). On multivariate analysis, a greater LV mass z-score and previous aortic valve balloon dilation were associated with a DFS of ≥2. In patients with catheterization data (n = 65), E/E' correlated with LV end-diastolic pressure. Those with a DFS of ≥2 had a greater LV end-diastolic pressure and mean pulmonary artery pressure than those with a DFS <2. In conclusion, DD is common in young patients with AS and AS+AR but not in those with pure AR. A greater LV mass and previous aortic valve dilation were associated with DD.

    View details for DOI 10.1016/j.amjcard.2012.09.026

    View details for Web of Science ID 000313607100016

    View details for PubMedID 23102884

  • Bare metal stenting for obstructed small diameter homograft conduits in the right ventricular outflow tract CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Carr, M., Bergersen, L., Marshall, A. C., Keane, J. F., Lock, J. E., Emani, S. M., McElhinney, D. B. 2013; 81 (1): E44-E52

    Abstract

    Our aim was to assess acute hemodynamic changes with stent insertion, outcomes, and factors associated with increased longevity of stented small diameter homograft conduits.Right ventricle-to-pulmonary artery (RV-PA) homograft conduits are commonly used to palliate RV outflow tract obstruction. Bare metal stenting (BMS) and transcatheter pulmonary valve implantation have been shown to relieve obstructed larger diameter conduits and may delay surgical conduit reintervention. Less is known about BMS of small conduits.From 1992 to 2009, BMS was performed to relieve obstruction in 106 homograft conduits that were ≤12 mm at implant. The peak RV-PA gradient fell from 54.3 ± 17.4 mm Hg at baseline to 46.1 ± 15.2 mm Hg after balloon dilation alone and to 25.1 ± 11.4 mm Hg with stenting (all P < 0.001). Higher pre-BMS gradient and RV pressure were the only factors associated with higher post-BMS RV-PA gradient (≥30 mm Hg; both P < 0.001). There were no procedural deaths, two patients required surgical removal of embolized stents. At a median follow-up of 1.6 years, 83 conduits were replaced; freedom from conduit reoperation after BMS was 66% ± 5% at 1 year and 28% ± 5% at 3 years. Factors associated with shorter freedom from reoperation included implanted conduit diameter <10 mm (P = 0.009), higher post-stent RV-PA gradient (P = 0.026), and higher post-stent RV pressure (P < 0.01); only post-stent RV pressure remained significant on multivariable analysis (P < 0.001).BMS was acutely effective for the treatment of obstructed small diameter homograft conduits, with low morbidity. Prolongation of small diameter homograft conduit longevity with BMS may be useful in the lifetime management of conduit dysfunction in this patient population.

    View details for DOI 10.1002/ccd.24369

    View details for Web of Science ID 000312942300007

    View details for PubMedID 22431478

  • Obstruction of the Superior Vena Cava After Neonatal Extracorporeal Membrane Oxygenation: Association With Chylothorax and Outcome of Transcatheter Treatment PEDIATRIC CRITICAL CARE MEDICINE Kazanci, S. Y., McElhinney, D. B., Thiagarajan, R., Bergersen, L., Wilson, J. M., Marshall, A. C., Lock, J. E., Mullen, M. P. 2013; 14 (1): 37-43

    Abstract

    Obstruction of the superior vena cava is one of the potential complications of neonatal extracorporeal membrane oxygenation. Chylothorax is a known complication of surgery involving the thoracic cavity in children, and of extracorporeal membrane oxygenation. The aim of this study was to evaluate the association between chylothorax and superior vena cava obstruction after neonatal extracorporeal membrane oxygenation.Twenty-two patients diagnosed with superior vena cava obstruction at ≤ 6 months of age (median 1.8 months) after neonatal extracorporeal membrane oxygenation were compared with a randomly selected cohort of 44 neonatal extracorporeal membrane oxygenation patients without superior vena cava obstruction. Among patients with superior vena cava obstruction, 18 underwent extracorporeal membrane oxygenation for respiratory disease and four for cardiac insufficiency. Chylothorax was more prevalent among patients with superior vena cava obstruction than controls (odds ratio 9.4 [2.2-40], p = .01) and was associated with extension of obstruction into the left innominate vein. Patients with superior vena cava obstruction were supported by extracorporeal membrane oxygenation for a longer duration than controls. Nineteen patients with superior vena cava obstruction (86%) underwent transcatheter balloon angioplasty and/or stent implantation (median 7 days after diagnosis), which decreased the superior vena cava pressure and superior vena cava-to-right atrium pressure gradient and increased the superior vena cava diameter (all p < 0.001). There were no serious procedural adverse events. Six study patients died within 30 days of the diagnosis of superior vena cava obstruction (including three of nine with chylothorax), which did not differ from controls. During a median follow-up of 2.7 yrs, two additional patients died and nine underwent 14 superior vena cava reinterventions.Among neonates treated with extracorporeal membrane oxygenation, superior vena cava obstruction is associated with an increased risk of chylothorax. In neonates with chylothorax after extracorporeal membrane oxygenation, evaluation for superior vena cava obstruction may be warranted. Although mortality is high in this population, transcatheter treatment can relieve superior vena cava obstruction and facilitate symptomatic improvement.

    View details for DOI 10.1097/PCC.0b013e31825b5270

    View details for Web of Science ID 000313352200011

    View details for PubMedID 23295835

  • Melody Valve Implant Within Failed Bioprosthetic Valves in the Pulmonary Position A Multicenter Experience CIRCULATION-CARDIOVASCULAR INTERVENTIONS Gillespie, M. J., Rome, J. J., Levi, D. S., Williams, R. J., Rhodes, J. F., Cheatham, J. P., Hellenbrand, W. E., Jones, T. K., Vincent, J. A., Zahn, E. M., McElhinney, D. B. 2012; 5 (6): 862-870

    Abstract

    Transcatheter pulmonary valve implantation using the Melody valve has emerged as an important therapy for the treatment of postoperative right ventricular outflow tract dysfunction. Melody-in-bioprosthetic valves (BPV) is currently considered an off-label indication. We review the combined experience with transcatheter pulmonary valve implantation within BPVs from 8 centers in the United States and discuss technical aspects of the Melody-in-BPV procedure.A total of 104 patients underwent Melody-in-BPV in the pulmonary position at 8 US centers from April 2007 to January 2012. Ten different types of BPVs were intervened on, with Melody valve implantation at the intended site in all patients. Following Melody valve implant, the peak right ventricle-to-pulmonary artery gradient decreased from 38.7 ± 16.3 to 10.9 ± 6.7 mm Hg (P<0.001), and the right ventricular systolic pressure fell from 71.6 ± 21.7 to 46.7 ± 15.9 mm Hg (P<0.001). There was no serious procedural morbidity, and no deaths related to the catheterization or implant. At a median follow-up of 12 months (1-46 months), no patients had more than mild regurgitation, and 4 had a mean right ventricular outflow tract gradient ≥30 mm Hg. During follow-up, there were 2 stent fractures, 3 cases of endocarditis (2 managed with surgical explant), and 2 deaths that were unrelated to the Melody valve.Transcatheter pulmonary valve implantation using the Melody valve within BPVs can be accomplished with a high rate of success, low procedure-related morbidity and mortality, and excellent short-term results. The findings of this preliminary multicenter experience suggest that the Melody valve is an effective transcatheter treatment option for failed BPVs.

    View details for DOI 10.1161/CIRCINTERVENTIONS.112.972216

    View details for Web of Science ID 000313576500026

    View details for PubMedID 23212395

  • Staged Left Ventricular Recruitment After Single-Ventricle Palliation in Patients With Borderline Left Heart Hypoplasia JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Emani, S. M., McElhinney, D. B., Tworetzky, W., Myers, P. O., Schroeder, B., Zurakowski, D., Pigula, F. A., Marx, G. R., Lock, J. E., del Nido, P. J. 2012; 60 (19): 1966-1974

    Abstract

    The goal of this study was to review results of a novel management strategy intended to rehabilitate the left heart (LH) in patients with LH hypoplasia who have undergone single-ventricle palliation (SVP).Management of patients with hypoplastic LH syndrome and borderline left ventricle (LV) involves 2 options: SVP or biventricular repair. We hypothesized that staged LV recruitment and biventricular conversion may be achieved after SVP by using a strategy consisting of relief of inflow and outflow tract obstructions, resection of endocardial fibroelastosis, and promotion of flow through the LV.Patients with hypoplastic LH and borderline LV who underwent traditional SVP (n = 34) or staged LV recruitment (n = 34) between 1995 and 2010 were retrospectively analyzed and compared with a control SVP group.Mean initial z-scores for LH structures before stage 1 SVP were not significantly different between groups. Mortality occurred in 4 of 34 patients after LV recruitment and in 7 of 34 after traditional SVP. LH dimension z-scores increased significantly over time after LV recruitment, whereas they declined after traditional SVP, with significant interaction between stage of palliation and treatment group. Restriction of the atrial septum (conducted in 19 of 34 patients) was the only predictor of increase in left ventricular end-diastolic volume (p < 0.001). Native biventricular circulation was achieved in 12 patients after staged LV recruitment; all of these patients had restriction at the atrial septum.In these patients with borderline LH disease who underwent SVP, it is possible to increase LH dimensions by using an LV recruitment strategy. In a subset of patients, this strategy allowed establishment of biventricular circulation.

    View details for DOI 10.1016/j.jacc.2012.07.041

    View details for Web of Science ID 000310352600019

    View details for PubMedID 23062531

  • Dilation of the Ascending Aorta After Balloon Valvuloplasty for Aortic Stenosis During Infancy and Childhood AMERICAN JOURNAL OF CARDIOLOGY McElhinney, D. B., Lacro, R. V., Gauvreau, K., O'Brien, C. M., Kazanci, S. Y., Vogel, M., Emani, S., Brown, D. W. 2012; 110 (5): 702-708

    Abstract

    Dilation of the ascending aorta (AA) is common in patients with a bicuspid aortic valve. The natural history of the aortic root and AA and the risk factors for dilation have not been characterized in patients with congenital aortic stenosis (AS) treated with balloon valvuloplasty during childhood. The present study was performed to determine the prevalence of aortic dilation in patients with congenital AS before and up to 20 years after balloon valvuloplasty performed during childhood. In patients who underwent balloon valvuloplasty for AS at age ≤ 18 years from 1984 to 2005, the aortic diameter measurements before intervention and at 5-year intervals afterward were recorded and the Z scores calculated. Among 156 patients (median age 1.5 years at valvuloplasty), the AA Z scores were significantly larger than normal before intervention (median Z score 1.5) and at all follow-up points (all p <0.001). Using mixed modeling, with time as a categorical variable (before intervention, 5-year window, 10-year window, and so forth), the mean AA Z score was greater at all postvalvuloplasty points than before the intervention, with mean Z score increases of 1.20 at 5 years and 2.11 at 20 years (p <0.001). Moderate or greater aortic regurgitation early after valvuloplasty was associated with greater AA Z scores than mild or less aortic regurgitation, with a progressive difference over time. More significant residual AS after valvuloplasty was associated with lower AA Z scores over time. In conclusion, AA dilation is common in children with congenital AS and continues to progress over many years after balloon valvuloplasty.

    View details for DOI 10.1016/j.amjcard.2012.04.053

    View details for Web of Science ID 000308618200015

    View details for PubMedID 22608951

  • Clinical and Stent-Related Outcomes After Transcatheter or Operative Placement of Bare-Metal Stents in the Ventricular Septum or Subvalvar Systemic Outflow Tract CIRCULATION-CARDIOVASCULAR INTERVENTIONS Porras, D., McElhinney, D. B., del Nido, P., Lock, J. E., Meadows, J., Marshall, A. C. 2012; 5 (4): 570-581

    Abstract

    Stenting of the systemic ventricular outflow tract and creation or enlargement of a ventricular septal defect using stents are potential therapeutic options in rare patients with congenital heart disease who develop significant ventricular hypertension due to outflow obstruction. These stents may be at increased risk of fracture because of exposure to cyclic compressive stresses. The objective of this study was to evaluate the safety and efficacy of this intervention and to determine the incidence of stent fracture and other adverse outcomes after placement of intraventricular or interventricular stents for this indication.Between 1992 and 2010, 27 patients underwent bare-metal stent placement in the ventricular septum or subvalvar systemic outflow tract, using 1 of the following 3 delivery approaches: (1) percutaneous (n=18), (2) intraoperative (n=8), and (3) hybrid (n=1). The median ventricular-to-aortic systolic pressure ratio decreased from 1.7 (1.3-2.6) to 1.1 (0.9-1.8) (P<0.001). Survival free from surgical reintervention for outflow obstruction was 92% at 1 year and 66% at 3 years. Stent fracture was diagnosed in 11 patients (41%) a median of 2.6 years (0.03-7.8 years) after stent placement and was always associated with recurrent obstruction. Survival free from diagnosis of stent fracture was 84% at 1 year and 73% at 3 years. Other adverse events included complete heart block (n=2) and increased atrioventricular valve regurgitation requiring surgical intervention (n=2).Transcatheter, intraoperative, or hybrid stenting of the ventricular septum or systemic outflow tract is feasible and effective in the short term. Stent fractures were common during follow-up and associated with recurrent obstruction.

    View details for DOI 10.1161/CIRCINTERVENTIONS.111.967190

    View details for Web of Science ID 000313575600021

    View details for PubMedID 22851527

  • Left Ventricular Remodeling and Improvement in Diastolic Function After Balloon Aortic Valvuloplasty for Congenital Aortic Stenosis CIRCULATION-CARDIOVASCULAR INTERVENTIONS Friedman, K. G., McElhinney, D. B., Colan, S. D., Porras, D., Powell, A. J., Lock, J. E., Brown, D. W. 2012; 5 (4): 549-554

    Abstract

    In congenital aortic stenosis, chronic pressure load has detrimental effects on left ventricular (LV) systolic and diastolic function. Reduction in LV pressure load with balloon aortic valvuloplasty (BAVP) may improve diastolic function.Echocardiographic and catheterization data for 25 consecutive patients undergoing BAVP for congenital aortic stenosis were retrospectively analyzed. Median age at BAVP was 11.5 years (3.2-40.1). LV end-diastolic pressure was elevated (≥15 mm Hg) in 72% of patients, with a median of 17 mm Hg (range, 9-24). With BAVP, median aortic stenosis gradient was reduced from 63 mm Hg (range, 44-105) to 30 mm Hg (range, 10-43). Aortic regurgitation increased from trivial (none to mild) to mild (trivial to moderate). Pre-BAVP early diastolic mitral inflow velocity/tissue Doppler early diastolic velocity (E/E´) correlated with LV end-diastolic pressure (r=0.52, P=0.007). On follow-up echocardiography (median, 11 months after BAVP), aortic stenosis gradient was lower (P<0.001) and degree of AR was higher (P=0.01) compared with pre-BAVP echocardiograms. LV end-diastolic volume z-score increased (P=0.02), LV mass was unchanged, and LV mass:volume decreased (P=0.002). Mitral annular and septal E´ (P<0.001) were higher and E/E´ was lower after dilation (10.8 versus 14.2, P<0.001). Lower pre-BAVP E/E´ and lower pre-BAVP LV mass z-score were associated with lower post-BAVP E/E.After BAVP, LV remodeling characterized by an increase in EDV and decrease in LV mass:volume occurs and echocardiographic measures of diastolic function and LV end-diastolic pressure improve in most patients. Risk factors for persistent diastolic dysfunction include higher pre-BAVP LV mass z-score and worse pre-BAVP diastolic function.

    View details for DOI 10.1161/CIRCINTERVENTIONS.112.968958

    View details for Web of Science ID 000313575600018

    View details for PubMedID 22739787

  • Left ventricular function and geometry in fetuses with severe tricuspid regurgitation ULTRASOUND IN OBSTETRICS & GYNECOLOGY Ishii, T., Tworetzky, W., Harrild, D. M., Marcus, E. N., McElhinney, D. B. 2012; 40 (1): 55-61

    Abstract

    Neonatal congenital tricuspid valve (TV) dysplasia and/or displacement (Ebstein's malformation) with severe tricuspid regurgitation (TR) is a challenging condition in which outcomes are frequently poor. Little is known about left ventricular (LV) function during the perinatal period in patients with congenital TV disease. The objective of this study was to evaluate LV function in fetuses with congenital TV anomalies associated with significant TR.Serial fetal echocardiograms in 16 fetuses with congenital TV dysplasia and/or displacement (five neonatal survivors and 11 fetal or neonatal deaths) were reviewed. LV stroke volume, LV end-diastolic volume (LVEDV), LV end-diastolic dimension (LVIDd), the LV eccentricity index, thoracic and cardiac areas and the cardiothoracic area ratio (CTAR), the right atrium area index, and LV longitudinal strains were compared according to gestational age and clinical outcome.The gestational age-adjusted LVEDV (Z-score) was lower in late gestation (-1.2 ± 1.2 at last examination ≥ 28 weeks) than earlier in gestation (0.3 ± 1.5 at last examination < 28 weeks) and LV output was lower than reported late-gestation normal values. LV short-axis dimension correlated with LV volume and CTAR. LV mid-septal strain was lower than the normal average of fetal mid-septal strain and correlated with the LV eccentricity index. Among these parameters, only the LV eccentricity index differed between survivors and non-survivors.LV function and anatomy are abnormal in fetuses with severe congenital TV anomalies and may be important contributors to outcome.

    View details for DOI 10.1002/uog.10115

    View details for Web of Science ID 000305822800008

    View details for PubMedID 21997973

  • Reliability and Accuracy of Echocardiographic Right Heart Evaluation in the US Melody Valve Investigational Trial JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Brown, D. W., McElhinney, D. B., Araoz, P. A., Zahn, E. M., Vincent, J. A., Cheatham, J. P., Jones, T. K., Hellenbrand, W. E., O'Leary, P. W. 2012; 25 (4): 383-U138

    Abstract

    Appropriate patient selection for transcatheter pulmonary valve (TPV) replacement requires accurate evaluation of right ventricular (RV) performance. The aim of this study was to evaluate the reliability and accuracy of echocardiography for evaluating RV parameters in patients in the five-center Melody TPV trial.Echocardiographic data were compared with cardiac magnetic resonance (CMR) and catheterization; interobserver comparisons were made using site and core laboratory data.Doppler echocardiographic assessments of RV outflow tract obstruction and RV pressure showed excellent interobserver agreement; mean Doppler gradients were correlated most closely with gradients at catheterization (R = 0.66), and Doppler RV pressure estimates were correlated well with catheterization data (R = 0.58). Assessment of pulmonary regurgitation (PR) using a three-point severity scale showed good agreement with CMR-derived PR fraction (86%). The tricuspid annular Z score was highly reproducible but correlated weakly with CMR RV end-diastolic volume (R = 0.21). However, RV apical diastolic area was highly reproducible (R = 0.87) and had an excellent correlation with CMR RV end-diastolic volume (R = 0.78); all patients with indexed RV apical diastolic areas ≥30 cm(2)/m(2) had CMR RV end-diastolic volumes ≥160 mL/m(2). RV function using the fractional area change method showed a fair correlation with CMR RV ejection fraction (R = 0.48).In patients with dysfunctional RV outflow tract conduits, echocardiography provided reproducible, accurate estimates of pressure overload and RV size. Echocardiographic assessment of PR correlated less closely with CMR PR fraction but showed good categorical agreement; assessment of RV function by these methods was suboptimal. Echocardiography alone may be a suitable screening test for some TPV replacement candidates; CMR may be indicated for TPV replacement decisions hinging on assessment of RV function.

    View details for DOI 10.1016/j.echo.2011.12.022

    View details for Web of Science ID 000302131900004

    View details for PubMedID 22265459

  • Outcomes After Stent Implantation for the Treatment of Congenital and Postoperative Pulmonary Vein Stenosis in Children CIRCULATION-CARDIOVASCULAR INTERVENTIONS Balasubramanian, S., Marshall, A. C., Gauvreau, K., Peng, L. F., Nugent, A. W., Lock, J. E., McElhinney, D. B. 2012; 5 (1): 109-117

    Abstract

    Pulmonary vein stenosis (PVS) is a rare condition that can lead to worsening pulmonary hypertension and cardiac failure in children, and it is frequently lethal. Surgical and transcatheter approaches are acutely successful but restenosis is common and rapid.We reviewed outcomes among patients who underwent transcatheter pulmonary vein stent implantation for congenital or postoperative PVS at <18 years of age. A total of 74 pulmonary veins were stented with bare metal, drug-eluting, or covered stents in 47 patients. Primary diagnoses included PVS associated with anomalous venous return in 51%, PVS associated with other congenital cardiovascular defects in 36%, and congenital ("de novo") PVS in 13% of patients. Median age at the time of pulmonary vein stent implantation was 1.4 years. During a median cross-sectional follow-up of 3.1 years, 21 patients died. Estimated survival was 62±8% at 1 year and 50±8% at 5 years after pulmonary vein stent implantation. Stent placement acutely relieved focal obstruction in all veins. Of the 54 stents reexamined with catheterization, 32 underwent reintervention. Freedom from reintervention was 62±7% at 6 months and 42±7% at 1 year. Stent occlusion was documented in 9 cases and significant in-stent stenosis in 17 cases. Stent implantation diameter ≥7 mm was associated with longer freedom from reintervention (hazard ratio, 0.32; P=0.015) and from significant in-stent stenosis (hazard ratio, 0.14; P=0.002). Major acute complications occurred in 5 cases.Transcatheter stent implantation can acutely relieve PVS in children, but reintervention is common. Larger stent lumen size at implantation is associated with longer stent patency and a lower risk of reintervention.

    View details for DOI 10.1161/CIRCINTERVENTIONS.111.964189

    View details for Web of Science ID 000300610900021

    View details for PubMedID 22253356

  • Cardiopulmonary exercise function among patients undergoing transcatheter pulmonary valve implantation in the US Melody valve investigational trial AMERICAN HEART JOURNAL Batra, A. S., McElhinney, D. B., Wang, W., Zakheim, R., Garofano, R. P., Daniels, C., Yung, D., Cooper, D. M., Rhodes, J. 2012; 163 (2): 280-287

    Abstract

    We assessed the hypothesis that there is an improvement in clinical and physiologic parameters of cardiopulmonary exercise testing (CPET) after implantation of a transcatheter pulmonary valve (TPV).Transcatheter pulmonary valve provides a new tool for treating conduit stenosis and regurgitation in patients with right ventricle (RV) to pulmonary artery conduit dysfunction.Patients who underwent a TPV placement between January 2007 and January 2010 (N = 150) were investigated with a standardized CPET protocol before and at 6 months after TPV placement. Cardiopulmonary exercise testing was performed on a mechanically braked cycle ergometer with respiratory gas exchange analysis.Six months post TPV, small but statistically significant improvements were observed in the maximum workload (65.0% ± 18.8% to 68.3% ± 20.3% predicted, P < .001) and the ratio of minute ventilation to CO(2) production at the anaerobic threshold (30.8 ± 4.7 to 29.1 ± 4.1, P < .001). There was no significant change in peak oxygen consumption (VO(2)). Patients with pre-TPV hemodynamics consistent with RV dysfunction and patients with a lower pre-TPV peak VO(2) tended to have the greatest improvement in peak VO(2). The correlation between TPV-related improvements in peak VO(2) and baseline clinical variables were weak, however, and these variables could not be used to reliably identify patients likely to have improved peak VO(2) after TPV.In patients with RV to pulmonary artery conduit dysfunction, TPV is associated with modest improvement in exercise capacity and gas exchange efficiency during exercise.

    View details for DOI 10.1016/j.ahj.2011.10.017

    View details for Web of Science ID 000300226600022

    View details for PubMedID 22305848

  • Stent Fracture, Valve Dysfunction, and Right Ventricular Outflow Tract Reintervention After Transcatheter Pulmonary Valve Implantation Patient-Related and Procedural Risk Factors in the US Melody Valve Trial CIRCULATION-CARDIOVASCULAR INTERVENTIONS McElhinney, D. B., Cheatham, J. P., Jones, T. K., Lock, J. E., Vincent, J. A., Zahn, E. M., Hellenbrand, W. E. 2011; 4 (6): 602-U130

    Abstract

    Among patients undergoing transcatheter pulmonary valve (TPV) replacement with the Melody valve, risk factors for Melody stent fracture (MSF) and right ventricular outflow tract (RVOT) reintervention have not been well defined.From January 2007 to January 2010, 150 patients (median age, 19 years) underwent TPV implantation in the Melody valve Investigational Device Exemption trial. Existing conduit stents from a prior catheterization were present in 37 patients (25%, fractured in 12); 1 or more new prestents were placed at the TPV implant catheterization in 51 patients. During follow-up (median, 30 months), MSF was diagnosed in 39 patients. Freedom from a diagnosis of MSF was 77±4% at 14 months (after the 1-year evaluation window) and 60±9% at 39 months (3-year window). On multivariable analysis, implant within an existing stent, new prestent, or bioprosthetic valve (combined variable) was associated with longer freedom from MSF (P<0.001), whereas TPV compression (P=0.01) and apposition to the anterior chest wall (P=0.02) were associated with shorter freedom from MSF. Freedom from RVOT reintervention was 86±4% at 27 months. Among patients with a MSF, freedom from RVOT reintervention after MSF diagnosis was 49±10% at 2 years. Factors associated with reintervention were similar to those for MSF.MSF was common after TPV implant in this multicenter experience and was more likely in patients with severely obstructed RVOT conduits and when the TPV was directly behind the anterior chest wall and/or clearly compressed. A TPV implant site protected by a prestent or bioprosthetic valve was associated with lower risk of MSF and reintervention.

    View details for DOI 10.1161/CIRCINTERVENTIONS.111.965616

    View details for Web of Science ID 000300549500012

    View details for PubMedID 22075927

  • Short-Term Performance of the Transcatheter Melody Valve in High-Pressure Hemodynamic Environments in the Pulmonary and Systemic Circulations CIRCULATION-CARDIOVASCULAR INTERVENTIONS Hasan, B. S., McElhinney, D. B., Brown, D. W., Cheatham, J. P., Vincent, J. A., Hellenbrand, W. E., Jones, T. K., Zahn, E. M., Lock, J. E. 2011; 4 (6): 615-620

    Abstract

    The Melody valve is approved for percutaneous pulmonary valve replacement in dysfunctional right ventricular outflow tracts. The function of this valve when subjected to high-pressure loads in humans is unknown. The aim of this study was to describe the immediate and short-term results of Melody valves implanted in a high-pressure environment.Definitions of a high-pressure system were established for Melody valves implanted in the systemic (ie, aortic or mitral position) and pulmonary (ie, right ventricular outflow tract conduit or tricuspid valve annulus) circulations. Implants in these environments were ascertained from databases of the 5 centers that participated in the US Investigational Device Exemption trial. Thirty implants met the inclusion criteria: 23 pulmonary circulation implants (all in the pulmonary position) systemic circulation implants (5 in the native aortic position, 1 in a left ventricle-to-descending aorta conduit, and 1 in the mitral annulus). All pulmonary circulation implants were performed percutaneously in the catheterization laboratory. A hybrid approach (surgical exposure for transcatheter implant) was used for 4 of the aortic implants. There were no procedure-related deaths. Three patients died of nonprocedure- and nonvalve-related causes. At 1 year, freedom from moderate to severe regurgitation was 100%, and freedom from mild regurgitation was 90%. Freedom from moderate to severe stenosis was 86% at 1 year.Short-term performance of the Melody valve in high-pressure environments is encouraging, with good valve function in all patients.

    View details for DOI 10.1161/CIRCINTERVENTIONS.111.963389

    View details for Web of Science ID 000300549500013

    View details for PubMedID 22075926

  • The evolving role of intraoperative balloon pulmonary valvuloplasty in valve-sparing repair of tetralogy of Fallot JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Robinson, J. D., Rathod, R. H., Brown, D. W., del Nido, P. J., Lock, J. E., McElhinney, D. B., Bacha, E. A., Marshall, A. C. 2011; 142 (6): 1367-1373

    Abstract

    The late morbidity of pulmonary regurgitation has intensified the interest in valve-sparing repair of tetralogy of Fallot. This study reviewed a single institution's experience with valve-sparing repair and investigated the role of intraoperative balloon valvuloplasty.A retrospective chart review identified 238 patients who underwent complete primary repair of tetralogy of Fallot at less than 180 days of age. Patients were divided into 4 groups on the basis of the type of right ventricular outflow tract repair: transannular patch (n = 111), commissurotomy or standard rigid dilation (n = 71), intraoperative balloon pulmonary valvuloplasty (n = 32), or no valvar intervention (n = 24).Baseline demographic and anatomic factors differed among the 4 procedural groups with substantial overlap. Among 142 patients with pulmonary valve hypoplasia (z score, -2 to -4), 37% had valve-sparing repair. These patients had significant annular growth over time: z score increased 0.67 and 1.00 per year in the intraoperative balloon valvuloplasty (P < .001) and traditional valve-sparing (P < .001) groups, respectively. Rates of valve growth did not differ across groups, but z scores were 0.58 lower for the balloon valvuloplasty group across all time points (P = .001). Freedom from reintervention and surgery was shorter for the balloon valvuloplasty group than for the other groups (P < .001).Patients with tetralogy of Fallot and pulmonary valve hypoplasia who undergo valve-sparing repair with intraoperative balloon valvuloplasty have significant longitudinal annular growth, with normalization of annular size over time. Despite application in patients with more hypoplastic valves, balloon valvuloplasty resulted in similar valve growth and pulmonary regurgitation as traditional methods, but higher rates of reintervention. Although the precise role of this technique needs further refinement, it is likely to be most useful in patients with moderate pulmonary stenosis and moderate pulmonary valve dysplasia.

    View details for DOI 10.1016/j.jtcvs.2011.02.047

    View details for Web of Science ID 000297214200011

    View details for PubMedID 21703641

  • Catheterization for Congenital Heart Disease Adjustment for Risk Method (CHARM) JACC-CARDIOVASCULAR INTERVENTIONS Bergersen, L., Gauvreau, K., Foerster, S. R., Marshall, A. C., McElhinney, D. B., Beekman, R. H., Hirsch, R., Kreutzer, J., Balzer, D., Vincent, J., Hellenbrand, W. E., Holzer, R., Cheatham, J. P., Moore, J. W., Burch, G., Armsby, L., Lock, J. E., Jenkins, K. J. 2011; 4 (9): 1037-1046

    Abstract

    This study sought to develop a method to adjust for case mix complexity in catheterization for congenital heart disease to allow equitable comparisons of adverse event (AE) rates.The C3PO (Congenital Cardiac Catheterization Project on Outcomes) has been prospectively collecting data using a Web-based data entry tool on all catheterization cases at 8 pediatric institutions since 2007.A multivariable logistic regression model with high-severity AE outcome was built using a random sample of 75% of cases in the multicenter cohort; the models were assessed in the remaining 25%. Model discrimination was assessed by the C-statistic and calibration with Hosmer-Lemeshow test. The final models were used to calculate standardized AE ratios.Between August 2007 and December 2009, 9,362 cases were recorded at 8 pediatric institutions of which high-severity events occurred in 454 cases (5%). Assessment of empirical data yielded 4 independent indicators of hemodynamic vulnerability. Final multivariable models included procedure type risk category (odds ratios [OR] for category: 2 = 2.4, 3 = 4.9, 4 = 7.6, all p < 0.001), number of hemodynamic indicators (OR for 1 indicator = 1.5, ≥2 = 1.8, p = 0.005 and p < 0.001), and age <1 year (OR: 1.3, p = 0.04), C-statistic 0.737, and Hosmer-Lemeshow test p = 0.74. Models performed well in the validation dataset, C-statistic 0.734. Institutional event rates ranged from 1.91% to 7.37% and standardized AE ratios ranged from 0.61 to 1.41.Using CHARM (Catheterization for Congenital Heart Disease Adjustment for Risk Method) to adjust for case mix complexity should allow comparisons of AE among institutions performing catheterization for congenital heart disease.

    View details for DOI 10.1016/j.jcin.2011.05.021

    View details for Web of Science ID 000295602800015

    View details for PubMedID 21939947

  • Echocardiographic Evaluation Before Bidirectional Glenn Operation in Functional Single-Ventricle Heart Disease Comparison to Catheter Angiography CIRCULATION-CARDIOVASCULAR IMAGING Stern, K. W., McElhinney, D. B., Gauvreau, K., Geva, T., Brown, D. W. 2011; 4 (5): 498-505

    Abstract

    Cardiac catheterization is routinely performed in patients with single ventricle before bidirectional Glenn operation (BDG). There is interest in noninvasive evaluation alone before BDG, but concern for echocardiography successfully imaging the relevant anatomy persists. We evaluated the accuracy of echocardiographic imaging of vascular anatomy.Diagnostic images of 130 patients who had echocardiography and catheterization before BDG were reviewed; diameters of the pulmonary arteries (PAs) and aortic arch were measured, and stenoses were recorded. Patient and procedural factors associated with echocardiographic imaging were analyzed. Median age at echocardiography was 4 months; the most common diagnosis was hypoplastic left heart syndrome (55%). The left PA was imaged by echocardiography in 83 patients (64%), with 4 of 21 stenoses (19%) diagnosed by catheterization identified; similarly, the right PA was imaged in 81 (62%), and 3 of 17 stenoses (18%) were identified. The distal aortic arch was visualized in 104 (80%), with successful identification of 21 of 27 (78%) of coarctations diagnosed by catheterization. Complete vascular echocardiography (visualization of PAs and aortic arch) occurred in 43% and was not obtained more frequently with sedation.In a large cohort of patients presenting for BDG, evaluation by echocardiography frequently failed to image the PAs and missed the majority of PA stenoses. Sedation did not appear to improve the performance of echocardiography for evaluation of the PAs. Echocardiography cannot be relied on as the sole investigation before BDG.

    View details for DOI 10.1161/CIRCIMAGING.110.963280

    View details for Web of Science ID 000295030600008

    View details for PubMedID 21730025

  • Factors Associated With Subsequent Arch Reintervention After Initial Balloon Aortoplasty in Patients With Norwood Procedure and Arch Obstruction JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Porras, D., Brown, D. W., Marshall, A. C., del Nido, P., Bacha, E. A., McElhinney, D. B. 2011; 58 (8): 868-876

    Abstract

    The aim of this study was to determine whether there are identifiable factors associated with increased risk for aortic arch reintervention in patients who have undergone balloon aortoplasty (BD) for aortic arch obstruction (COA) after the Norwood procedure (NP).BD has been shown to be an effective acute therapy for COA after the NP. However, recurrent obstruction requiring repeat intervention is not uncommon.All patients who underwent BD as the initial intervention for COA after the NP from to January 1993 to May 2009 were retrospectively analyzed (n = 116).The median age at initial BD was 4.5 months. The median follow-up period was 3.4 years. Procedures were considered acutely successful in 92% of patients, with a median gradient reduction overall from 24 to 3 mm Hg (p < 0.0001) and a COA diameter increase of 52% (p < 0.0001). By Kaplan-Meier analysis, freedom from reintervention was 69% at 1 year and 58% at 5 years, and freedom from reoperation was 82% at 1 year and 79% at 5 years. By Cox regression analysis, proximal arch obstruction, age <3 months at BD, moderate or greater ventricular dysfunction, moderate or greater atrioventricular valve regurgitation on pre-catheterization echocardiography, and higher post-BD coarctation gradient were associated with shorter freedom from reoperation.Despite a high acute success rate, a significant proportion of patients treated with BD for post-NP COA underwent reintervention during follow-up. The risk for arch reintervention is highest in patients with proximal arch obstruction, those age <3 months at the time of BD, and those with less successful acute results.

    View details for DOI 10.1016/j.jacc.2010.12.050

    View details for Web of Science ID 000293729600014

    View details for PubMedID 21835324

  • Postnatal Left Ventricular Diastolic Function After Fetal Aortic Valvuloplasty AMERICAN JOURNAL OF CARDIOLOGY Friedman, K. G., Margossian, R., Graham, D. A., Harrild, D. M., Emani, S. M., Wilkins-Haug, L. E., McElhinney, D. B., Tworetzky, W. 2011; 108 (4): 556-560

    Abstract

    Fetal aortic balloon valvuloplasty (FAV) has shown promise in altering in utero progression of aortic stenosis to hypoplastic left heart syndrome. In patients who achieve a biventricular circulation after FAV, left ventricular (LV) compliance may be impaired. Echocardiographic indexes of diastolic function were compared between patients with biventricular circulation after FAV, congenital aortic stenosis (AS), and age-matched controls. In the neonatal period, patients with FAV had similar LV, aortic, and mitral valve dimensions but more evidence of endocardial fibroelastosis than patients with AS. Patients with FAV underwent more postnatal cardiac interventions than patients with AS (p = 0.007). Mitral annular early diastolic tissue velocity (E') was lower in patients with FAV and those with AS and controls in the neonatal period and over follow-up (p <0.001). Septal E' was similar among all 3 groups in the neonatal period. In follow-up patients, with FAV had lower septal E' than patients with AS or controls (p <0.001). Early mitral inflow velocity/E' was higher in patients with FAV as neonates and at follow-up (p <0.001). Mitral inflow pulse-wave Doppler-derived indexes of diastolic function were similar between groups. In conclusion, echocardiographic evidence of LV diastolic dysfunction is common in patients with biventricular circulation after FAV and persists in short-term follow-up. LV diastolic dysfunction in this unique population may have important implications on long-term risk of left atrial and subsequent pulmonary hypertension.

    View details for DOI 10.1016/j.amjcard.2011.03.085

    View details for Web of Science ID 000294040700013

    View details for PubMedID 21624551

  • Percutaneous Tricuspid Valve Replacement in Congenital and Acquired Heart Disease JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Roberts, P. A., Boudjemline, Y., Cheatham, J. P., Eicken, A., Ewert, P., McElhinney, D. B., Hill, S. L., Berger, F., Khan, D., Schranz, D., Hess, J., Ezekowitz, M. D., Celermajer, D., Zahn, E. 2011; 58 (2): 117-122

    Abstract

    This study sought to describe the first human series of percutaneous tricuspid valve replacements in patients with congenital or acquired tricuspid valve (TV) disease.Percutaneous transcatheter heart valve replacement of the ventriculoarterial (aortic, pulmonary) valves is established. Although there are isolated reports of transcatheter atrioventricular heart valve replacement (hybrid and percutaneous), this procedure has been less frequently described; we are aware of no series describing this procedure for TV disease.We approached institutions with significant experience with the Melody percutaneous pulmonary valve (Medtronic, Inc., Minneapolis, Minnesota) to collect data where this valve had been implanted in the tricuspid position. Clinical and procedural data were gathered for 15 patients. Indications for intervention included severe hemodynamic compromise and perceived high surgical risk; all had prior TV surgery and significant stenosis and/or regurgitation of a bioprosthetic TV or a right atrium-to-right ventricle conduit.Procedural success was achieved in all 15 patients. In patients with predominantly stenosis, mean tricuspid gradient was reduced from 12.9 to 3.9 mm Hg (p < 0.01). In all patients, tricuspid regurgitation was reduced to mild or none. New York Heart Association functional class improved in 12 patients. The only major procedural complication was of third-degree heart block requiring pacemaker insertion in 1 patient. One patient developed endocarditis 2 months after implant, and 1 patient with pre-procedural multiorgan failure did not improve and died 20 days after the procedure. The remaining patients have well-functioning Melody valves in the TV position a median of 4 months after implantation.In selected cases, patients with prior TV surgery may be candidates for percutaneous TV replacement.

    View details for DOI 10.1016/j.jacc.2011.01.044

    View details for Web of Science ID 000292189300003

    View details for PubMedID 21718905

  • Early Reintervention on the Pulmonary Arteries and Right Ventricular Outflow Tract After Neonatal or Early Infant Repair of Truncus Arteriosus Using Homograft Conduits AMERICAN JOURNAL OF CARDIOLOGY Lund, A. M., Vogel, M., Marshall, A. C., Emani, S. M., Pigula, F. A., Tworetzky, W., McElhinney, D. B. 2011; 108 (1): 106-113

    Abstract

    Patients with truncus arteriosus often require pulmonary arterial (PA) and/or right ventricular outflow tract (RVOT) reintervention within the first year of repair. However, little is known about the risk factors for early reintervention on the PAs or RVOT in this population. The objective of the present retrospective cohort study was to determine the risk factors for early PA or RVOT reintervention after repair of truncus arteriosus in neonates and young infants. Of 156 patients ascertained (median age at repair 14 days; 143 early survivors), reinterventions on the RVOT and/or PAs were performed in 109. The first reintervention was catheter therapy in 73 patients (conduit dilation/stenting in 29, PA dilation/stenting in 31, both in 13) and conduit reoperation in 36 patients. The freedom from any RVOT or PA reintervention was 68 ± 4% at 1 year and 48 ± 5% at 2 years. The factors associated with early reintervention (shorter 1-year freedom from reintervention) on univariate analysis were repair quartile, neonatal repair, smaller weight at repair, and smaller implanted conduit size. On multivariable analysis, only smaller conduit size remained significant (multivariable hazard ratio 0.66/mm, range 0.53 to 0.83; p <0.001). The freedom from conduit reoperation was 92 ± 3% at 1 year and 76 ± 4% at 2 years. Overall, the left and right PA sizes were modestly larger than normal by the 1-sample t test, and PA Z scores and the PA area index were not associated with the risk of reintervention. Early reintervention for PA and/or RVOT conduit obstruction is common after neonatal and early infant repair of truncus arteriosus using homograft conduits. A smaller conduit size was associated with early RVOT/PA reintervention. The branch PA size was normal before surgery, suggesting that the PA stenosis in these patients resulted from factors other than intrinsic stenosis or hypoplasia.

    View details for DOI 10.1016/j.amjcard.2011.03.006

    View details for Web of Science ID 000292785600018

    View details for PubMedID 21530938

  • Cavopulmonary pathway modification in patients with heterotaxy and newly diagnosed or persistent pulmonary arteriovenous malformations after a modified Fontan operation JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY McElhinney, D. B., Marx, G. R., Marshall, A. C., Mayer, J. E., del Nido, P. J. 2011; 141 (6): 1362-U57

    Abstract

    Pulmonary arteriovenous malformations are an important but uncommon complication of cavopulmonary connection, particularly in patients with heterotaxy. Absence of hepatic venous effluent in pulmonary arterial blood seems to be a predisposing factor. Pulmonary arteriovenous malformations are most common after superior cavopulmonary anastomosis, but may develop, progress, or persist in 1 lung after Fontan completion if hepatic venous blood streams completely or primarily to the contralateral lung.Among 53 patients with heterotaxy and inferior vena cava interruption who underwent a modified Fontan procedure from 1985 to 2005, 8 had unilateral streaming of hepatic venous flow and clinically significant pulmonary arteriovenous malformations after hepatic venous inclusion and underwent reconfiguration of the cavopulmonary pathway. In all 8 patients, the hepatic vein-pulmonary artery pathway was contralateral to and offset from the pulmonary artery anastomosis of the single or dominant superior vena cava. Pathway reconfiguration included pulmonary arterial stenting (n = 2), revision of the superior vena cava-pulmonary artery connection (n = 1), construction of a branched hepatic vein-pulmonary artery conduit (n = 2), and surgical or transcatheter construction of a direct hepatic vein-azygous vein pathway (n = 5).Hepatic vein-azygous vein connection led to improvement in 4 of 5 patients; other approaches typically did not lead to improvement.Resolution of hypoxemia after cavopulmonary pathway reconfiguration in patients with unilateral pulmonary arteriovenous malformations and hepatic venous flow-streaming after Fontan completion supports the importance of hepatic venous effluent in the pathogenesis of pulmonary arteriovenous malformations and the practice of cavopulmonary pathway revision in such patients. Completion or reconfiguration of the Fontan circulation with direct hepatic vein-azygous vein connection may provide the most reliable mixing and bilateral distribution of hepatic venous blood in this population of patients.

    View details for DOI 10.1016/j.jtcvs.2010.08.088

    View details for Web of Science ID 000290710900011

    View details for PubMedID 21146835

  • Assessment of Left Ventricular Endocardial Fibroelastosis in Fetuses With Aortic Stenosis and Evolving Hypoplastic Left Heart Syndrome AMERICAN JOURNAL OF CARDIOLOGY McElhinney, D. B., Vogel, M., Benson, C. B., Marshall, A. C., Wilkins-Haug, L. E., Silva, V., Tworetzky, W. 2010; 106 (12): 1792-1797

    Abstract

    Systematic evaluation of left ventricular (LV) endocardial fibroelastosis (EFE) in the fetus has not been reported. The role of EFE in the pre- and postnatal evolution of hypoplastic left heart disease, and the implications of EFE for outcomes after prenatal intervention for fetal aortic stenosis with evolving hypoplastic left heart syndrome have also not been determined. A 4-point grading system (0-3) was devised for the assessment of fetal LV echogenicity, which was presumed to be due to EFE. Two reviewers independently graded EFE on the preintervention echocardiograms of fetuses treated with in utero aortic valvuloplasty for evolving hypoplastic left heart syndrome from 2000 to 2008. Intra- and interobserver reproducibility was determined for the EFE grade and characterization of related echocardiographic features. The relations among EFE severity, other left heart anatomic and physiologic variables, and postintervention outcomes were analyzed. The assessment and grading of EFE was possible for both observers in all 74 fetuses studied. By consensus, the EFE severity was grade 1 in 31 patients, grade 2 in 32, and grade 3 in 11. Fetuses with mild (grade 1) EFE had significantly greater maximum instantaneous aortic stenosis gradients (e.g., higher LV pressures) and less globular LV geometry than patients with grade 2 or 3 EFE on preintervention echocardiogram. The severity of EFE was not associated with the size of the aortic valve or LV. From preintervention to late gestation, the time-indexed change in LV end-diastolic volume was significantly greater in fetuses with grade 1 EFE than those with more severe EFE. Incorporation of EFE severity into our previously published threshold score improved the sensitivity and positive predictive value for the postnatal biventricular outcomes. In conclusion, echocardiographic grading of EFE is possible, with reasonable intra- and interobserver reliability in midgestation fetuses with evolving hypoplastic left heart syndrome. EFE severity corresponded to some indexes of left heart size, geometry, and function and with the probability of a biventricular outcome postnatally. Additional experience and external validation of the EFE grading scoring system are necessary.

    View details for DOI 10.1016/j.amjcard.2010.08.022

    View details for Web of Science ID 000285735000020

    View details for PubMedID 21126622

  • Sudden Unexpected Death After Balloon Valvuloplasty for Congenital Aortic Stenosis JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Brown, D. W., Dipilato, A. E., Chong, E. C., Gauvreau, K., McElhinney, D. B., Colan, S. D., Lock, J. E. 2010; 56 (23): 1939-1946

    Abstract

    The aims of this study were to determine the incidence and risk factors of sudden unexpected death (SUD) after balloon aortic valvuloplasty (BAVP) for congenital aortic stenosis (AS) and to assess the effect of exercise restriction.Exercise restriction is recommended for some patients with congenital AS because of a perceived increased risk for SUD. Little is known about the incidence of SUD in those with treated AS or the efficacy of exercise restriction in preventing SUD.A review was conducted of 528 patients who underwent BAVP for congenital AS at Children's Hospital Boston from 1984 to 2008. Exercise restriction status was ascertained for those ≥4 years of age, censored at aortic valve replacement or transplantation.Median subsequent follow-up was 12.0 years (range 0 to 24.8 years), for a total of 6,344 patient-years of follow-up. There were 63 deaths, with SUD in 6 patients, 5 of which occurred at ≤18 months of age. For patients ≥4 years of age at most recent follow-up with no histories of pulmonary hypertension (n = 422), median follow-up after BAVP was 14.6 years, for 6,019 patient-years of follow-up. Exercise restriction was prescribed in 183 patients (43%; 2,541 patient-years) and no restriction in 220 (52%; 2,691 patient-years); there were insufficient data in 19 patients. There were 17 deaths in this cohort of 422 patients, with 1 SUD (the patient, who was exercise restricted, died during sleep), for an incidence of 0.18/1,000 patient-years (95% confidence interval: 0.01 of 1,000 to 1.01 of 1,000).SUD is extremely rare after BAVP for congenital AS. No beneficial effect of the recommendation for exercise restriction was observed in this longitudinal cohort with 6,000 patient-years of follow-up.

    View details for DOI 10.1016/j.jacc.2010.06.048

    View details for Web of Science ID 000284571800008

    View details for PubMedID 21109118

  • What is the Clinical Utility of Routine Cardiac Catheterization Before a Fontan Operation? PEDIATRIC CARDIOLOGY Banka, P., McElhinney, D. B., Bacha, E. A., Mayer, J. E., Gauvreau, K., Geva, T., Brown, D. W. 2010; 31 (7): 977-985

    Abstract

    Patients with single-ventricle circulation presenting for Fontan completion routinely undergo cardiac catheterization despite ongoing debate concerning its additive value. Increasing interest in noninvasive preoperative evaluation alone led the authors to analyze the utility of routine pre-Fontan catheterization and to determine whether a subset of patients could avoid this invasive procedure. Patients younger than 5 years referred for pre-Fontan evaluation were retrospectively reviewed. Medical records and catheter angiograms were examined, and catheterizations were categorized as "additive" based on predetermined criteria. Associations between precatheterization variables, catheterization findings, and short-term postoperative outcomes were evaluated. Cardiac catheterization was clinically nonadditive for 89 of 175 patients undergoing pre-Fontan evaluation (51%). There were no robust precatheterization predictors of a nonadditive catheterization. Echocardiography did not fully demonstrate the relevant anatomy of 115 patients (66%), most frequently due to inadequate visualization of the pulmonary arteries, and 22 patients had additive catheterizations due to new diagnostic findings alone. Interventions at catheterization were frequent and deemed "important" for 64 patients (37%). Catheterization hemodynamic data were not associated with early postoperative outcomes. Minor catheterization complications occurred for 51 patients (29%) and major complications for 4 patients (2%). Although at least 50% of the patients presenting for Fontan completion may be able to avoid routine catheterization safely, an echocardiography-based imaging strategy alone is insufficient to allow proper identification of those who could be evaluated noninvasively. A more comprehensive imaging strategy not based solely on echocardiography should be considered.

    View details for DOI 10.1007/s00246-010-9736-3

    View details for Web of Science ID 000282424800006

    View details for PubMedID 20503042

  • Short- and Medium-Term Outcomes After Transcatheter Pulmonary Valve Placement in the Expanded Multicenter US Melody Valve Trial CIRCULATION McElhinney, D. B., Hellenbrand, W. E., Zahn, E. M., Jones, T. K., Cheatham, J. P., Lock, J. E., Vincent, J. A. 2010; 122 (5): 507-U132

    Abstract

    Transcatheter pulmonary valve placement is an emerging therapy for pulmonary regurgitation and right ventricular outflow tract obstruction in selected patients. The Melody valve was recently approved in the United States for placement in dysfunctional right ventricular outflow tract conduits.From January 2007 to August 2009, 136 patients (median age, 19 years) underwent catheterization for intended Melody valve implantation at 5 centers. Implantation was attempted in 124 patients; in the other 12, transcatheter pulmonary valve placement was not attempted because of the risk of coronary artery compression (n=6) or other clinical or protocol contraindications. There was 1 death from intracranial hemorrhage after coronary artery dissection, and 1 valve was explanted after conduit rupture. The median peak right ventricular outflow tract gradient was 37 mm Hg before implantation and 12 mm Hg immediately after implantation. Before implantation, pulmonary regurgitation was moderate or severe in 92 patients (81% with data); no patient had more than mild pulmonary regurgitation early after implantation or during follow-up (>or=1 year in 65 patients). Freedom from diagnosis of stent fracture was 77.8+/-4.3% at 14 months. Freedom from Melody valve dysfunction or reintervention was 93.5+/-2.4% at 1 year. A higher right ventricular outflow tract gradient at discharge (P=0.003) and younger age (P=0.01) were associated with shorter freedom from dysfunction.In this updated report from the multicenter US Melody valve trial, we demonstrated an ongoing high rate of procedural success and encouraging short-term valve function. All reinterventions in this series were for right ventricular outflow tract obstruction, highlighting the importance of patient selection, adequate relief of obstruction, and measures to prevent and manage stent fracture. Clinical Trial Registration- URL: http://www.clinicaltrials.gov. Unique identifier: NCT00740870.

    View details for DOI 10.1161/CIRCULATIONAHA.109.921692

    View details for Web of Science ID 000280561100010

    View details for PubMedID 20644013

  • To Close or Not to Close: The Very Small Patent Ductus Arteriosus CONGENITAL HEART DISEASE Fortescue, E. B., Lock, J. E., Galvin, T., McElhinney, D. B. 2010; 5 (4): 354-365

    Abstract

    Patent ductus arteriosus (PDA) accounts for approximately 10% of all congenital heart diseases, with an incidence of at least 2-4 per 1000 term births. Closure of the large, hemodynamically significant PDA is established as the standard of care, and can be performed safely and effectively using either surgical or transcatheter methods. The appropriate management of the very small, hemodynamically insignificant PDA is less clear. Routine closure of such defects has been advocated to eliminate or reduce the risk of infective endocarditis (IE). However, the risk of IE in patients with a small PDA appears to be extremely low, and IE is treatable. Although closure of the small PDA is generally safe and technically successful, it is unknown whether this treatment truly improves the risk:benefit balance compared with observation. In this article, we review the published literature on the natural history and treatment outcomes in individuals with a PDA, the epidemiology and outcomes of IE, particularly in association with PDA, and the rationale and evidence for closure of the very small PDA.

    View details for DOI 10.1111/j.1747-0803.2010.00435.x

    View details for Web of Science ID 000289419800005

    View details for PubMedID 20653702

  • Comparison of Conventional and Cutting Balloon Angioplasty for Congenital and Postoperative Pulmonary Vein Stenosis in Infants and Young Children CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Peng, L. F., Lock, J. E., Nugent, A. W., Jenkins, K. J., McElhinney, D. B. 2010; 75 (7): 1084-1090

    Abstract

    Pulmonary vein stenosis (PVS) is a rare and often lethal condition in children. The optimal treatment for congenital and postoperative PVS is unknown.We compared outcomes of conventional balloon angioplasty performed for PVS from 1999 to 2003 against cutting balloon angioplasty performed from 2004 to 2007. A total of 100 previously undilated pulmonary veins in 54 patients were studied: 48 veins dilated with conventional balloons and 52 with cutting balloons. Acute results included significantly reduced gradients and increased lumen diameters with both treatments. Acutely, cutting balloon angioplasty and conventional angioplasty yielded similar relative reduction of the PVS gradient (median 78% vs. 63%, P = 0.08) and increase in lumen diameter (median 77% vs. 59%, P = 0.07). There was one procedural death of a critically ill infant, and four cardiac arrests, but no adverse events necessitating surgical intervention. Survival free from reintervention was poor in both groups, and shorter in the cutting balloon group (73% at 1 month, 11% at 6 months, and 4% at 1 year) than in the conventional angioplasty group (77% at 1 month, 35% at 6 months, and 23% at 1 year; P = 0.01).Both conventional and cutting balloon angioplasty were effective at decreasing gradient and increasing lumen size acutely in patients with congenital and postoperative PVS, but reintervention was common with both treatments. Both methods of angioplasty provided limited benefit, and neither was curative for this complex disease.

    View details for DOI 10.1002/ccd.22405

    View details for Web of Science ID 000278503900022

    View details for PubMedID 20146341

  • Current Status of Fetal Cardiac Intervention CIRCULATION McElhinney, D. B., Tworetzky, W., Lock, J. E. 2010; 121 (10): 1256-1263
  • Long-Term Pulmonary Regurgitation Following Balloon Valvuloplasty for Pulmonary Stenosis Risk Factors and Relationship to Exercise Capacity and Ventricular Volume and Function JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Harrild, D. M., Powell, A. J., Trang, T. X., Geva, T., Lock, J. E., Rhodes, J., McElhinney, D. B. 2010; 55 (10): 1041-1047

    Abstract

    This study sought to examine the prevalence and predictors of pulmonary regurgitation (PR) following balloon dilation (BD) for pulmonary stenosis (PS) and to investigate its impact on ventricular volume and function, and exercise tolerance.Balloon pulmonary valvuloplasty relieves PS but can cause late PR. The sequelae of isolated PR are not well understood.Patients were at least 7 years of age and 5 years removed from BD, and had no other form of congenital heart disease or significant residual PS. Cardiac magnetic resonance imaging and exercise testing were performed prospectively to quantify PR fraction, ventricular volumes and function, and exercise capacity.Forty-one patients underwent testing a median of 13.1 years after BD. The median PR fraction was 10%; 14 patients (34%) had PR fraction >15%; 7 (17%) had PR >30%. PR fraction was associated with age at dilation (ln-transformed, R = -0.47, p = 0.002) and balloon:annulus ratio (R = 0.57, p < 0.001). The mean right ventricular (RV) end-diastolic volume z-score was 1.8 +/- 1.9; RV dilation (z-score > or =2) was present in 14/35 patients (40%). PR fraction correlated closely with indexed RV end-diastolic volume (R = 0.79, p < 0.001) and modestly with RV ejection fraction (R = 0.50, p < 0.001). Overall, peak oxygen consumption (Vo(2)) (% predicted) was below average (92 +/- 17%, p = 0.006). Patients with PR fraction >15% had significantly lower peak Vo(2) than those with less PR (85 +/- 17% vs. 96 +/- 16%, p = 0.03).Mild PR and RV dilation are common in the long term following BD. A PR fraction >15% is associated with lower peak Vo(2), suggesting that isolated PR and consequent RV dilation are related to impaired exercise cardiopulmonary function.

    View details for DOI 10.1016/j.jacc.2010.01.016

    View details for Web of Science ID 000275143200013

    View details for PubMedID 20202522

  • Significance and outcome of left heart hypoplasia in fetal congenital diaphragmatic hernia ULTRASOUND IN OBSTETRICS & GYNECOLOGY Vogel, M., McElhinney, D. B., Marcus, E., Morash, D., Jennings, R. W., Tworetzky, W. 2010; 35 (3): 310-317

    Abstract

    In patients with a left-sided congenital diaphragmatic hernia (CDH), the left ventricle (LV) is often compressed and smaller than normal. The objective of this study was to investigate whether small left heart dimensions prenatally normalize after birth in patients with CDH, or whether prenatal indices of left heart size and flow predict postnatal outcome.Clinical and echocardiographic data were reviewed for patients diagnosed with left-sided CDH prenatally. Cardiac dimensions and flows were compared with normative data. Among liveborn patients, pre- and postnatal Z-scores of left heart structures were compared, and associations between prenatal indices and outcome were assessed.Of 125 patients diagnosed prenatally with CDH, 111 had a left-sided defect. Of these, 85 were liveborn, including 20 with congenital heart disease. Gestational age-adjusted dimensions of fetal left heart structures, including aortic valve diameter, mitral valve (MV) diameter, LV long-axis, LV short-axis and LV volume, were all smaller than normal (P < 0.001). On average, the LV contributed 33 +/- 8% of combined ventricular output, lower than the normal 40-50%. Z-scores of left heart structures increased from the prenatal echocardiogram to the postnatal study, with average changes ranging from 0.56 +/- 1.68 (aortic valve) to 1.39 +/- 1.85 (LV volume). Among liveborn patients, there was no association between prenatal left heart Z-scores and postnatal survival.Hypoplasia of and reduced flow through the left heart are common among fetuses with CDH. After birth and CDH repair, left heart dimensions generally normalize, with adequate size to support a biventricular circulation, even when there is very low flow through the left heart in mid- and late-gestation.

    View details for DOI 10.1002/uog.7497

    View details for Web of Science ID 000276134800011

    View details for PubMedID 20143332

  • Fetal Diagnosis of Interrupted Aortic Arch AMERICAN JOURNAL OF CARDIOLOGY Vogel, M., Vernon, M. M., McElhinney, D. B., Brown, D. W., Colan, S. D., Tworetzky, W. 2010; 105 (5): 727-734

    Abstract

    To determine the frequency of prenatal detection among liveborn patients with an interrupted aortic arch (IAA), the accuracy of prenatal diagnosis, and the anatomic features associated with IAA in the fetus. The prenatal diagnosis of an IAA is challenging. The data on the features and outcomes of fetal IAA are limited. This was a retrospective review of the fetuses and neonates diagnosed with IAA at the Children's Hospital Boston. From 1988 to 2009, 26 fetuses were diagnosed with an IAA. Of these, 21 were live born, and 5 pregnancies were terminated. Of these 21 patients, 18 were confirmed to have an IAA after birth and 3 had severe aortic coarctation. Of the 56 patients diagnosed with an IAA as neonates, 3 had a prenatal echocardiogram that did not include the correct diagnosis. Among the liveborn patients with a postnatally confirmed IAA, 24% were diagnosed prenatally, which increased from 11% during the first 7-year period to 43% more recently. Also, 15% of the prenatally diagnosed patients with IAA had a family history of structural or genetic anomalies. In fetuses with an IAA, echocardiographic Z-scores for the aortic valve and ascending aorta were significantly lower than in normal fetuses, but the left ventricular dimensions were normal. Aortopulmonary diameter ratios were abnormally low. In conclusion, although the identification of IAA on a prenatal echocardiogram can be challenging, a number of anatomic features can facilitate the diagnosis. In particular, a low aortopulmonary diameter ratio in the absence of a ventricular size discrepancy should prompt consideration of this diagnosis. Despite the diagnostic challenges, the frequency of prenatal diagnosis of the IAA is increasing.

    View details for DOI 10.1016/j.amjcard.2009.10.053

    View details for Web of Science ID 000278137100025

    View details for PubMedID 20185024

  • Serial Cardiopulmonary Exercise Testing in Patients with Previous Fontan Surgery PEDIATRIC CARDIOLOGY Fernandes, S. M., McElhinney, D. B., Khairy, P., Graham, D. A., Landzberg, M. J., Rhodes, J. 2010; 31 (2): 175-180

    Abstract

    Patients with previous Fontan surgery have reduced peak oxygen consumption (Vo 2), and data regarding progression of exercise intolerance is limited. The purpose of this study was to assess the evolution of exercise tolerance in patients with previous Fontan surgery. We performed a retrospective cohort study of patients with previous Fontan surgery who underwent cardiopulmonary exercise testing between November 2002 and January 2009. Patients were required to have completed at least two tests, with adequate levels of effort, >or-6 months apart. We identified 78 patients (55% male) who had undergone a total of 215 cardiopulmonary exercise tests. Age at initial study was 19.7 +/- 10.2 years; time interval since previous Fontan surgery 13.3 +/- 5.7 years; and time interval between the initial and the most recent exercise test was 3.0 +/- 1.4 years. Morphologic left ventricle was present in 58 (74.4%) patients. At baseline, peak Vo 2 averaged 24.7 +/- 7.0 ml/kg/min (63.7% +/- 15.8% predicted). At most recent study, peak Vo 2 averaged 23.2 +/- 7.2 ml/kg/min, (60.4% +/- 13.9% predicted). Percent predicted peak Vo 2 declined slowly, with a mean rate of decline of 1.25 +/- 0.36 percentage points/y (p < 0.001). Most of the decline occurred in patients < 18 years of age (mean rate of decline 1.78 +/- 0.46 percentage points/y; p = 0.0004). Thereafter, the rate of decline was less marked (mean rate of decline 0.54 +/- 0.57 percentage points/y; p = not significant). Ventricular morphology, type of Fontan procedure, and cardiac medications were not predictive of progressive exercise intolerance. In conclusion, although the exercise function of previous Fontan patients tends to decline during late adolescence, it appears to stabilize during early adulthood.

    View details for DOI 10.1007/s00246-009-9580-5

    View details for Web of Science ID 000274333900001

    View details for PubMedID 19915891

  • Reversible Ductus Arteriosus Constriction due to Maternal Indomethacin after Fetal Intervention for Hypoplastic Left Heart Syndrome with Intact/Restrictive Atrial Septum FETAL DIAGNOSIS AND THERAPY Vogel, M., Wilkins-Haug, L. E., McElhinney, D. B., Marshall, A. C., Benson, C. B., Silva, V., Tworetzky, W. 2010; 27 (1): 40-45

    Abstract

    Fetal cardiac intervention (FCI) has been performed at our center in selected fetuses with complex congenital heart disease since 2000. Most interventions are performed in fetuses with a ductus arteriosus (DA)-dependent circulation. Indomethacin promotes closure of the DA in newborns and in fetal life, a potentially life threatening complication in fetuses with ductus-dependent congenital heart disease.We reviewed our experience with FCI with a focus on the frequency, features, and clinical course of ductal constriction. Fetuses undergoing FCI receive comprehensive pre- and postoperative cardiac and cerebral ultrasound evaluation, approximately 24 hours before and after the procedure, including imaging of DA flow and Doppler assessment of the umbilical artery and vein, ductus venosus, and, since 2004, the middle cerebral artery.Among 113 fetuses that underwent FCI, 24 of which were older than 28 0/7 weeks gestation, 2 were found to have DA constriction due to indomethacin therapy within 24 hours of intervention. Both of these were 30-week fetuses with hypoplastic left heart syndrome and restrictive or intact atrial septum. The DA was stenotic by spectral and color Doppler, and middle cerebral and umbilical artery pulsatility indexes were depressed. After discontinuation of indomethacin, the Doppler indices improved or normalized.Close echocardiographic monitoring of fetal Doppler flow velocities is very important after fetal intervention and indomethacin treatment, as the consequences of DA constriction in a fetus with hypoplastic left heart syndrome are potentially lethal. Sonographic evaluation should include measurement of cerebral and umbilical arterial flow velocities as well as color and spectral Doppler interrogation of the DA.

    View details for DOI 10.1159/000268290

    View details for Web of Science ID 000274051800007

    View details for PubMedID 20016136

  • CEREBRAL BLOOD FLOW CHARACTERISTICS AND BIOMETRY IN FETUSES UNDERGOING PRENATAL INTERVENTION FOR AORTIC STENOSIS WITH EVOLVING HYPOPLASTIC LEFT HEART SYNDROME ULTRASOUND IN MEDICINE AND BIOLOGY McElhinney, D. B., Benson, C. B., Brown, D. W., Wilkins-Haug, L. E., Marshall, A. C., Zaccagnini, L., Tworetzky, W. 2010; 36 (1): 29-37

    Abstract

    Children with hypoplastic left heart syndrome (HLHS) are at risk for neurodevelopmental dysfunction; prenatal factors may play a role in this predilection. Cerebral blood flow profiles are abnormal in fetuses with HLHS, raising the possibility that cerebral hemodynamics in utero may be related to neurodevelopmental abnormalities. Prenatal aortic valvuloplasty for fetal aortic stenosis with evolving HLHS is technically feasible and improves left heart hemodynamics. This study aimed to assess the effects of prenatal intervention on cerebral blood flow profiles and head circumference in fetuses with evolving HLHS. Seventy fetuses underwent prenatal aortic valvuloplasty for evolving HLHS (median 23 weeks gestation). Among 46 fetuses that had successful valvuloplasty and available data, middle cerebral artery (MCA) pulsatility (PI) and resistive (RI) indices were abnormal (Z-scores -1.7+/-1.1 and -2.2+/-1.4, p<0.001). Early post-valvuloplasty (n=33) and at late gestation follow-up (n=28), MCA PI and RI Z-scores remained low with no difference from pre- or early postintervention. Fetal head circumference was normal, as were umbilical artery PI and RI Z-scores. Cerebral blood flow characteristics are abnormal in mid-gestation fetuses with evolving HLHS, suggesting low cerebral vascular impedance. The mechanisms and significance of these abnormalities are unknown. Prenatal aortic valvuloplasty did not have a major impact on these indices. (E-mail: doff.mcelhinney@cardio.chboston.org).

    View details for DOI 10.1016/j.ultrasmedbio.2009.09.004

    View details for Web of Science ID 000278012200005

    View details for PubMedID 19931971

  • Primary left ventricular rehabilitation is effective in maintaining two-ventricle physiology in the borderline left heart JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Emani, S. M., Bacha, E. A., McElhinney, D. B., Marx, G. R., Tworetzky, W., Pigula, F. A., del Nido, P. J. 2009; 138 (6): 1276-1282

    Abstract

    Borderline left heart disease is characterized by left heart obstructive lesions (coarctation, aortic and mitral stenoses, left ventricular hypoplasia) and endocardial fibroelastosis. The multilevel obstruction and impaired left ventricular systolic and diastolic function contribute to failure of biventricular circulation. We studied the effects of left ventricular rehabilitation--endocardial fibroelastosis resection with mitral or aortic valvuloplasty--on left ventricular function and clinical outcomes.All patients with borderline left heart structures and endocardial fibroelastosis who underwent a primary left ventricular rehabilitation procedure were retrospectively analyzed to determine operative mortality, reintervention rates, and hemodynamic status. Left heart dimensions and hemodynamics were recorded from preoperative and postoperative echocardiogram and cardiac catheterization. Postoperative left atrial pressure was obtained from the intracardiac line early after left ventricular rehabilitation. Preoperative and postoperative values were compared by paired t test.Between 1999 and 2008, 9 patients with endocardial fibroelastosis and borderline left heart disease underwent left ventricular rehabilitation at a median age of 5.6 months (range, 1-38 months). There was no operative mortality, and at a median follow-up of 25 months (6 months to 10 years) there was 1 death from noncardiac causes and 2 patients required reoperations. Significant increases in ejection fraction and left ventricular end-diastolic volume were observed, whereas left atrial pressure and right ventricular/left ventricular pressure ratios decreased postoperatively.In patients with borderline left hearts, primary left ventricular rehabilitation with endocardial fibroelastosis resection and mitral and aortic valvuloplasty results in improved left ventricular systolic and diastolic performance and decreased right ventricular pressures. This approach may provide an alternative to single-ventricle management in this difficult patient group.

    View details for DOI 10.1016/j.jtcvs.2009.08.009

    View details for Web of Science ID 000272029800002

    View details for PubMedID 19819471

  • Implantation of the Melody Transcatheter Pulmonary Valve in Patients With a Dysfunctional Right Ventricular Outflow Tract Conduit Early Results From the US Clinical Trial JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Zahn, E. M., Hellenbrand, W. E., Lock, J. E., McElhinney, D. B. 2009; 54 (18): 1722-1729

    Abstract

    This study was designed to evaluate the safety, procedural success, and short-term effectiveness of the Melody transcatheter pulmonary valve (Medtronic, Inc., Minneapolis, Minnesota) in patients with dysfunctional right ventricular outflow tract conduits.Conduit dysfunction has recently been treated with transcatheter pulmonary valve placement. There have been no prospective, multicenter trials evaluating this technology.Standardized entry criteria, implantation, and follow-up protocols were used. Nonimplanting core laboratories were used to evaluate results.Between January 2007 and September 2007, 34 patients underwent catheterization for intended Melody valve implantation at 3 centers. Mean age was 19.4 +/- 7.7 years. Initial conduit Doppler mean gradient was 28.8 +/- 10.1 mm Hg, and 94% of patients had moderate or severe pulmonary regurgitation (PR). Implantation was successful in 29 of 30 attempts and not attempted in 4 patients. Procedural complications included conduit rupture requiring urgent surgery and device removal (n = 1), wide-complex tachycardia (n = 1), and distal pulmonary artery guidewire perforation (n = 1). Peak systolic conduit gradient fell acutely from 37.2 +/- 16.3 mm Hg to 17.3 +/- 7.3 mm Hg, and no patient had more than mild PR. There were no deaths or further device explants. At 6-month follow-up, conduit Doppler mean gradient was 22.4 +/- 8.1 mm Hg, and PR fraction by magnetic resonance imaging was significantly improved (3.3 +/- 3.6% vs. 27.6 +/- 13.3%, p < 0.0001). Stent fracture occurred in 8 of 29 implants; 3 of these were treated with a second Melody valve for recurrent stenosis later in follow-up.Implantation of the Melody valve for right ventricular outflow tract conduit dysfunction can be performed by experienced operators at multiple centers, appears safe, and has encouraging acute and short-term outcomes.

    View details for DOI 10.1016/j.jacc.2009.06.034

    View details for Web of Science ID 000270971400011

    View details for PubMedID 19850214

  • Predictors of Technical Success and Postnatal Biventricular Outcome After In Utero Aortic Valvuloplasty for Aortic Stenosis With Evolving Hypoplastic Left Heart Syndrome CIRCULATION McElhinney, D. B., Marshall, A. C., Wilkins-Haug, L. E., Brown, D. W., Benson, C. B., Silva, V., Marx, G. R., Mizrahi-Arnaud, A., Lock, J. E., Tworetzky, W. 2009; 120 (15): 1482-U48

    Abstract

    Aortic stenosis in the midgestation fetus with a normal-sized or dilated left ventricle predictably progresses to hypoplastic left heart syndrome when associated with certain physiological findings. Prenatal balloon aortic valvuloplasty may improve left heart growth and function, possibly preventing evolution to hypoplastic left heart syndrome.Between March 2000 and October 2008, 70 fetuses underwent attempted aortic valvuloplasty for critical aortic stenosis with evolving hypoplastic left heart syndrome. We analyzed this experience to determine factors associated with procedural and postnatal outcome. The median gestational age at intervention was 23 weeks. The procedure was technically successful in 52 fetuses (74%). Relative to 21 untreated comparison fetuses, subsequent prenatal growth of the aortic and mitral valves, but not the left ventricle, was improved after intervention. Nine pregnancies (13%) did not reach a viable term or preterm birth. Seventeen patients had a biventricular circulation postnatally, 15 from birth. Larger left heart structures and higher left ventricular pressure at the time of intervention were associated with biventricular outcome. A multivariable threshold scoring system was able to discriminate fetuses with a biventricular outcome with 100% sensitivity and modest positive predictive value.Technically successful aortic valvuloplasty alters left heart valvar growth in fetuses with aortic stenosis and evolving hypoplastic left heart syndrome and, in a subset of cases, appeared to contribute to a biventricular outcome after birth. Fetal aortic valvuloplasty carries a risk of fetal demise. Fetuses undergoing in utero aortic valvuloplasty with an unfavorable multivariable threshold score at the time of intervention are very unlikely to achieve a biventricular circulation postnatally.

    View details for DOI 10.1161/CIRCULATIONAHA.109.848994

    View details for Web of Science ID 000270706500005

    View details for PubMedID 19786635

  • In Utero Valvuloplasty for Pulmonary Atresia With Hypoplastic Right Ventricle: Techniques and Outcomes PEDIATRICS Tworetzky, W., McElhinney, D. B., Marx, G. R., Benson, C. B., Brusseau, R., Morash, D., Wilkins-Haug, L. E., Lock, J. E., Marshall, A. C. 2009; 124 (3): E510-E518

    Abstract

    Prenatal intervention for fetuses with pulmonary atresia with an intact ventricular septum (PA/IVS) has the potential to alter right heart physiologic features in utero, facilitating right heart growth and improving the prospect of a biventricular outcome after birth.Since 2002, we have considered prenatal intervention for fetal PA/IVS in patients with (1) membranous pulmonary atresia, with identifiable pulmonary valve (PV) leaflets or membrane; (2) an intact or highly restrictive ventricular septum; and (3) right heart hypoplasia, with a tricuspid valve annulus z score of -2 or below and an identifiable but small right ventricle. Intervention was performed through direct cardiac puncture under ultrasound guidance, with percutaneous access or access through a limited laparotomy.Ten fetuses underwent attempted balloon dilation of the PV in utero. The first 4 procedures were technically unsuccessful, and the most-recent 6 were technically successful. Compared with control fetuses with PA/IVS who did not undergo prenatal intervention and had univentricular outcomes after birth, the tricuspid valve annulus, right ventricle length, and PV annulus grew significantly more from midgestation to late gestation in the 6 fetuses who underwent successful interventions.In utero perforation and dilation of the PV in midgestation fetuses with PA/IVS is technically feasible and may be associated with improved right heart growth and postnatal outcomes for fetuses with moderate right heart hypoplasia in midgestation. There is an important learning curve for this procedure, and much remains to be learned about the selection of appropriate fetuses for prenatal intervention.

    View details for DOI 10.1542/peds.2008-2014

    View details for Web of Science ID 000269383100042

    View details for PubMedID 19706566

  • Exercise Function of Children with Congenital Aortic Stenosis Following Aortic Valvuloplasty during Early Infancy CONGENITAL HEART DISEASE Kipps, A. K., McElhinney, D. B., Kane, J., Rhodes, J. 2009; 4 (4): 258-264

    Abstract

    The objectives of this study were to characterize the exercise function of patients treated with balloon aortic valvuloplasty at 6 years old with a history of neonatal AS to undergo exercise testing.We enrolled 30 patients (median age 13.1 years) who underwent balloon aortic valvuloplasty at a median age of 12 days. At time of exercise testing, the median maximum Doppler AS gradient was 34 mm Hg (0-70 mm Hg); 11 patients had moderate or severe aortic regurgitation. All patients were asymptomatic. Overall, peak oxygen consumption (VO(2)) was below normal (87 +/- 18% predicted; P < .001), and was severely depressed (

    View details for DOI 10.1111/j.1747-0803.2009.00304.x

    View details for Web of Science ID 000207893700007

    View details for PubMedID 19664028

    View details for PubMedCentralID PMC4269337

  • Effectiveness of Balloon Valvuloplasty for Palliation of Mitral Stenosis After Repair of Atrioventricular Canal Defects AMERICAN JOURNAL OF CARDIOLOGY Robinson, J. D., Marx, G. R., del Nido, P. J., Lock, J. E., McElhinney, D. B. 2009; 103 (12): 1770-1773

    Abstract

    Closure of a mitral valve (MV) cleft, small left-sided cardiac structures, and ventricular imbalance all may contribute to mitral stenosis (MS) after repair of atrioventricular canal (AVC) defects. MV replacement is the traditional therapy but carries high risk in young children. The utility of balloon mitral valvuloplasty (BMV) in postoperative MS is not established and may offer alternative therapy or palliation. Since 1996, 10 patients with repaired AVC defects have undergone BMV at a median age of 2.5 years (range 8 months to 14 years), a median of 2 years after AVC repair. At catheterization, the median value of mean MS gradients was 16 mm Hg (range 12 to 22) and was reduced by 34% after BMV. Before BMV, there was mild mitral regurgitation in 9 of 10 patients, which increased to severe in 1 patient. All patients were alive at follow-up (median 5.4 years). Repeat BMV was performed in 4 patients, 10 weeks to 18 months after initial BMV. One patient underwent surgical valvuloplasty; 3 underwent MV replacement 2, 3, and 28 months after BMV. In the 6 patients (60%) with a native MV at most recent follow-up (median 3.2 years), the mean Doppler MS gradient was 9 mm Hg, the median weight had doubled, and weight percentile had increased significantly. In conclusion, BMV provides relief of MS in most patients with repaired AVC defects; marked increases in mitral regurgitation are uncommon. Because BMV can incompletely relieve obstruction and increase mitral regurgitation, it will not be definitive in most patients but will usually delay MV replacement to accommodate a larger prosthesis.

    View details for DOI 10.1016/j.amjcard.2009.02.062

    View details for Web of Science ID 000267407000025

    View details for PubMedID 19539091

  • Truncus Arteriosus: Diagnostic Accuracy, Outcomes, and Impact of Prenatal Diagnosis PEDIATRIC CARDIOLOGY Swanson, T. M., Tierney, E. S., Tworetzky, W., Pigula, F., McElhinney, D. B. 2009; 30 (3): 256-261

    Abstract

    Limited data exist on the impact of prenatal diagnosis and outcomes of fetal truncus arteriosus (TA). We sought to assess prenatal diagnostic accuracy and prenatal outcomes in fetuses with TA and compare postnatal outcomes in neonates with prenatally and postnatally diagnosed TA. Records were reviewed for patients diagnosed with TA in utero or at

    View details for DOI 10.1007/s00246-008-9328-7

    View details for Web of Science ID 000264884500007

    View details for PubMedID 19015910

  • Ultra-High-Pressure Balloon Angioplasty for Treatment of Resistant Stenoses Within or Adjacent to Previously Implanted Pulmonary Arterial Stents CIRCULATION-CARDIOVASCULAR INTERVENTIONS Maglione, J., Bergersen, L., Lock, J. E., McElhinney, D. B. 2009; 2 (1): 52-58

    Abstract

    Stents are essential tools in the management of pulmonary arterial (PA) stenosis in patients with congenital heart disease. Although stents can usually be reexpanded as children grow, resistant in-stent or peri-stent obstruction can complicate the management of PA stents. Angioplasty with ultra-high-pressure (UHP) balloons may facilitate successful treatment of stent-associated PA stenoses that are resistant to high-pressure dilation.We reviewed patients who underwent UHP angioplasty of in-stent or peri-stent PA stenoses that were resistant to high-pressure redilation. A resistant stenosis was defined as a residual balloon waist during high-pressure redilation of the stent, along with a pressure gradient and/or angiographic stenosis. Thirty-four lesions in 29 patients, including 8 with multiple concentric, overlapping, or adjacent stents, were included. The median age at UHP angioplasty was 9 years, and a median of 4 years had elapsed since unsuccessful high-pressure angioplasty. Thirty-one of the 34 (91% [81% to 100%]) UHP angioplasty procedures were successful in relieving the resistant stenosis. Balloon:waist diameter ratios were conservative (median 1.26), reflecting the ability of UHP balloons to "fracture" nearly all obstructions. After UHP dilation, lesion diameter increased by a median of 3.1 mm (36%), significantly more than after previous high-pressure dilation (1.3 mm, 19%; P<0.001). In 5 lesions, UHP angioplasty fractured the stent, allowing further vessel expansion. There were no vascular or other complications.UHP angioplasty was safe and effective for treatment of stent-related resistant PA stenosis in this series; the ability to fracture maximally expanded stents may extend the utility of stents in the pediatric population.

    View details for DOI 10.1161/CIRCINTERVENTIONS.108.826263

    View details for Web of Science ID 000276048500009

    View details for PubMedID 20031693

  • Results of in utero atrial septoplasty in fetuses with hypoplastic left heart syndrome PRENATAL DIAGNOSIS Marshall, A. C., Levine, J., Morash, D., Silva, V., Lock, J. E., Benson, C. B., Wilkins-Haug, L. E., McElhinney, D. B., Tworetzky, W. 2008; 28 (11): 1023-1028

    Abstract

    Neonates with hypoplastic left heart syndrome and intact or highly restrictive atrial septum have a high rate of mortality. We sought to assess the effect of prenatal intervention intended to create atrial septal defects in fetuses with this diagnosis.We reviewed the medical records and imaging of all fetuses undergoing intervention for atrial septal defect creation in the setting of hypoplastic left heart syndrome and intact atrial septum. The procedures were performed with a percutaneous cardiac puncture under the guidance of ultrasonography. For the 21 interventions, patient and procedural characteristics were analyzed to identify predictors of neonatal outcome.Of 21 procedures attempted between 24 and 34 weeks' gestation, 19 were technically successful. Fetal demise occurred in two cases. The size of the created defect varied and measured at least 3 mm in six fetuses. Among 19 neonates, a larger atrial septal defect was associated with higher oxygen saturation and less need for intervention prior to surgical single-ventricle palliation.Technically successful atrial septal defect creation in fetuses with hypoplastic left heart syndrome and intact atrial septum results in atrial septal defects of varying size; defects of at least 3 mm in diameter appear to confer postnatal benefit.

    View details for DOI 10.1002/pd.2114

    View details for Web of Science ID 000261125700007

    View details for PubMedID 18925607

  • Mitral valve replacement in infants and children 5 years of age or younger: Evolution in practice and outcome over three decades with a focus on supra-annular prosthesis implantation JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Tierney, E. S., Pigula, F. A., Berul, C. I., Lock, J. E., del Nido, P. J., McElhinney, D. B. 2008; 136 (4): 954-U8

    Abstract

    Successful mitral valve replacement in young children is limited by the lack of small prosthetic valves. Supra-annular prosthesis implantation can facilitate mitral valve replacement with a larger prosthesis in children with a small annulus, but little is known about its effect on the outcomes of mitral valve replacement in young children.One hundred eighteen children underwent mitral valve replacement at 5 years of age or younger from 1976-2006. Mitral valve replacement was supra-annular in 37 (32%) patients.Survival was 74% +/- 4% at 1 year and 56% +/- 5% at 10 years but improved over time (10-year survival of 83% +/- 7% from 1994-2006). Factors associated with worse survival included earlier mitral valve replacement date, age less than 1 year, complete atrioventricular canal, and additional procedures at mitral valve replacement, but not supra-annular mitral valve replacement. As survival improved during our more recent experience, the risks of supra-annular mitral valve replacement became apparent; survival was worse among patients with a supra-annular prosthesis after 1991. A pacemaker was placed in 18 (15%) patients within 1 month of mitral valve replacement and was less likely in patients who had undergone supra-annular mitral valve replacement. Among early survivors, freedom from redo mitral valve replacement was 72% +/- 5% at 5 years and 45% +/- 7% at 10 years. Twenty-one patients with a supra-annular prosthesis underwent redo mitral valve replacement. The second prosthesis was annular in 15 of these patients and upsized in all but 1, but 5 required pacemaker placement for heart block.Supra-annular mitral valve replacement was associated with worse survival than annular mitral valve replacement in our recent experience. Patients with supra-annular mitral valve replacement were less likely to have operative complete heart block but remained at risk when the prosthesis was subsequently replaced.

    View details for DOI 10.1016/j.jtcvs.2007.12.076

    View details for Web of Science ID 000260314800023

    View details for PubMedID 18954636

  • In situ fracture of stents implanted for relief of pulmonary arterial stenosis in patients with congenitally malformed hearts CARDIOLOGY IN THE YOUNG McElhinney, D. B., Bergersen, L., Marshall, A. C. 2008; 18 (4): 405-414

    Abstract

    One of the most common uses of stents in patients with congenitally malformed hearts is treatment of pulmonary arterial stenosis. Although there are reports of fractured pulmonary arterial stents, little is known about the risk factors for, and implications of, such fractures.We reviewed angiograms to identify fractures in stents previously inserted to relieve stenoses in pulmonary arteries from 1990 through 2001 in patients who also underwent follow-up catheterization at least 3 years after placement of the stent. We undertook matched cohort analysis, matching a ratio of 2 fractured to 1 unfractured stent.Overall, 166 stents meeting the criterions of our study had been placed in 120 patients. We identified fractures in 35 stents (21%) in 29 patients. All fractured stents were in the central pulmonary arteries, 24 (69%) in the central part of the right pulmonary artery, and all were complete axial fractures, or complex fractures along at least 2 planes. Stent-related factors associated with increased risk of fracture identified by multivariable logistic regression included placement in close apposition to the ascending aorta (p = 0.001), and a larger expanded diameter (p = 0.002). There was obstruction across 28 of 35 fractured stents, which was severe in 11. We re-stented 21 of the fractured stents, and recurrent fracture was later diagnosed in 3 of these. A fragment of the fractured stent embolized distally in 2 patients, without clinically important effects.In situ fracture of pulmonary arterial stents is relatively common, and in most cases is related to compression by the aorta. There is usually recurrent obstruction across the fractured stent, but fractured stents rarely embolize, and are not associated with other significant complications.

    View details for DOI 10.1017/S1047951108002424

    View details for Web of Science ID 000258393300009

    View details for PubMedID 18559137

  • Echocardiographic predictors of mitral stenosis-related death or intervention in infants AMERICAN HEART JOURNAL Tierney, E. S., Graham, D. A., McElhinney, D. B., Trevey, S., Freed, M. D., Colan, S. D., Geva, T. 2008; 156 (2): 384-390

    Abstract

    The purpose of this study is to identify independent echocardiographic predictors of mitral stenosis (MS)-related death or intervention in infants. Congenital MS is a rare and morphologically heterogeneous lesion with variable prognosis. Among patients diagnosed with MS in early infancy, echocardiographic factors associated with MS-related intervention or death have not been determined.The clinical and echocardiographic data of patients diagnosed with MS at age <6 months by echocardiography between 1986 and 2004 were reviewed. The primary outcome was a composite end point of either mitral valve (MV) intervention (catheter or surgery) or death related to MS. Multiple variables from the initial echocardiogram were analyzed for association with outcomes. Seventy-one patients (median age at diagnosis 63 days) fulfilled the inclusion criteria. Multivariate analysis identified higher initial MV mean inflow gradient (P = .009) and lower left ventricular (LV) diastolic length Z-score (P = .006) at presentation as predictors of intervention or death. Among patients with an initial MV inflow gradient < 2 mm Hg, none reached an end point, whereas, among patients with an initial mean gradient >/= 5.5 mm Hg, the risk of intervention or death was 85%. Among patients with a gradient > 2 and < 5.5 mm Hg, an end point was reached in 38%, and an LV diastolic length Z-score

    View details for DOI 10.1016/j.ahj.2008.03.019

    View details for Web of Science ID 000258333400034

    View details for PubMedID 18657675

  • Outcome of fetuses and infants with double inlet single left ventricle AMERICAN JOURNAL OF CARDIOLOGY Tham, E. B., Wald, R., McElhinney, D. B., Hirji, A., Goff, D., del Nido, P. J., Hornberger, L. K., Nield, L. E., Tworetzky, W. 2008; 101 (11): 1652-1656

    Abstract

    Double-inlet left ventricle (DILV) includes a diverse range of anatomic variables that affect the surgical strategy. The aim of this study was to determine the impact of anatomic subtype, associated anomalies, and fetal diagnosis on the management and outcomes of fetuses and infants with DILV. The outcomes of fetuses and infants with DILV diagnosed from 1990 to 2004 at 3 major referral centers were reviewed. Sixty-five cases of DILV were detected prenatally. Twenty-one of these pregnancies were terminated, including 17 of 37 (46%) in which the diagnoses were made at < or =24 weeks of gestation. An additional 106 patients were diagnosed with DILV within the first 3 months of life. The percentage of patients diagnosed prenatally increased significantly over the study period. Transplantation-free survival was 88%, 82%, 79%, and 76% at 1 month, 1 year, 5 years, and 10 years, respectively. Factors associated with improved survival in univariate analysis included year of birth after 1994, no neonatal Norwood or Damus procedure, and no neonatal surgery at all. In multivariate analysis, any neonatal surgery was the only factor associated with worse survival. Associated anomalies and prenatal diagnosis were not associated with postnatal outcome. In conclusion, although the frequency of prenatal diagnosis of DILV has increased significantly over the past 15 years, prenatal diagnosis is not associated with better postnatal survival. During this same period, postnatal survival has improved substantially. Neonatal surgery of any type was the only independent risk factor for worse survival.

    View details for DOI 10.1016/j.amjcard.2008.01.048

    View details for Web of Science ID 000256450700020

    View details for PubMedID 18489946

  • Importance of totally anomalous pulmonary venous connection and postoperative pulmonary vein stenosis in outcomes of heterotaxy syndrome PEDIATRIC CARDIOLOGY Foerster, S. R., Gauvreau, K., McElhinney, D. B., Geva, T. 2008; 29 (3): 536-544

    Abstract

    Historically, outcomes of patients with heterotaxy syndrome and congenital heart disease have been poor. Published series include patients treated over many decades or focus on specific patient/operative subgroups. This study was performed to evaluate midterm outcomes and determine anatomic risk factors for death in patients with all types of heterotaxy syndrome treated after 1985. We reviewed all infants with heterotaxy born between 1985 and 1997, presenting to one institution at <6 months age, followed for > or =5 years. Of 102 study patients (46 with asplenia phenotype, 56 with polysplenia phenotype), 48 (47%) died at a median age of 0.6 months, 12 without intervention. Survivors were followed for a median of 12.8 years (5-21.7 years). Independent predictors of mortality included obstructed totally anomalous pulmonary venous connection (TAPVC) (OR, 7.8; 95% CI, 1.9-32.9; p = 0.005), mild or greater atrioventricular (AV) valve regurgitation at presentation (OR, 3.5; 95% CI, 1.0-12.1; p = 0.03), and common AV canal (OR, 3.1; 95% CI, 1.1-8.5; p = 0.03). Sixteen patients developed pulmonary vein stenosis at a median age of 2 months, with 5 (31%) alive at follow-up. In patients with TAPVC, the mean indexed sum of pulmonary vein diameters was lower among patients who died than in survivors (42.3 +/- 8.3 vs 49.5 +/- 10.1 mm/m2; p = 0.02). Mortality remains high among patients with heterotaxy treated in the modern surgical era, particularly those with obstructed TAPVC. Pulmonary vein stenosis is common after repair of TAPVC in patients with heterotaxy, may be diagnosed beyond infancy, and is associated with poor outcomes.

    View details for DOI 10.1007/s00246-007-9128-5

    View details for Web of Science ID 000255754100010

    View details for PubMedID 18004616

  • Surgical aortic valvuloplasty in children and adolescents with aortic regurgitation: Acute and intermediate effects on aortic valve function and left ventricular dimensions JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Bacha, E. A., McElhinney, D. B., Guleserian, K. J., Colan, S. D., Jonas, R. A., del Nido, P. J., Marx, G. R. 2008; 135 (3): 552-U38

    Abstract

    Surgical aortic valvuloplasty is increasingly employed in the management of children and adolescents with aortic regurgitation, but the durability of this approach and factors associated with outcome are not well defined.From 1989 to 2005, a total of 81 patients younger than 19 years with moderate or severe aortic regurgitation underwent surgical aortic valvuloplasty. Aortic regurgitation was congenital in 20 cases, after treatment of aortic stenosis in 30, from other injuries to the aortic valve in 12, and from other causes in 19. Eighteen patients had moderate or severe aortic stenosis. Preoperative left ventricular end-diastolic dimension z score was 4.9 +/- 2.7.After surgical aortic valvuloplasty with various techniques, including pericardial leaflet augmentation in 80%, aortic regurgitation was improved in 77 patients and was mild or less in 68. Ten of 18 patients with moderate or severe aortic stenosis before repair had a decrease to mild, whereas 2 had progression from mild to moderate. Left ventricular end-diastolic dimension z score decreased by 2.9 +/- 2.1 (P < .001). During follow-up (median 4.7 years), 33 patients underwent aortic valve reinterventions, including aortic valve replacement in 25. Estimated freedoms from aortic valve replacement were 72% +/- 6% at 5 years and 54% +/- 9% at 7.5 years and were shorter in patients with moderate or severe aortic stenosis before surgical aortic valvuloplasty. Among surviving patients who did not undergo aortic valve replacement, aortic regurgitation at follow-up was moderate in 21 and trivial or mild in 34; left ventricular and aortic root dimensions were preserved.Surgical aortic valvuloplasty is a valid option with good intermediate results for children and adolescents with aortic regurgitation from a variety of causes, particularly for patients with less than moderate aortic stenosis.

    View details for DOI 10.1016/j.jtcvs.2007.09.057

    View details for Web of Science ID 000253860300015

    View details for PubMedID 18329469

  • Changes in left heart hemodynamics after technically successful in-utero aortic valvuloplasty ULTRASOUND IN OBSTETRICS & GYNECOLOGY Tierney, E. S., Wald, R. M., McElhinney, D. B., Marshall, A. C., Benson, C. B., Colan, S. D., Marcus, E. N., Marx, G. R., Levine, J. C., Wilkins-Haug, L., Lock, J. E., Tworetzky, W. 2007; 30 (5): 715-720

    Abstract

    Severe aortic stenosis in the mid-gestation fetus can progress to hypoplastic left heart syndrome (HLHS). @ In-utero aortic valvuloplasty is an innovative therapy to promote left ventricular growth and function and potentially to prevent HLHS. This study evaluated the effects of mid-gestation fetal balloon aortic valvuloplasty on subsequent fetal left ventricular function and left heart Doppler characteristics.We reviewed fetuses with aortic stenosis that underwent attempted in-utero aortic valvuloplasty between 2000 and 2006. Pre-intervention and the latest post-intervention fetal echocardiograms were analyzed to characterize changes in left heart function and Doppler characteristics in utero.Forty-two fetuses underwent attempted aortic valvuloplasty during the study period, 12 of which were excluded from analysis secondary to inadequate follow-up data, termination or fetal demise. Study fetuses (n = 30) underwent pre-intervention echocardiography at a median gestational age of 23 weeks, and were followed for a median of 66 +/- 23 days post-intervention. In 26 fetuses, aortic valvuloplasty was technically successful. Among these 26, left heart physiology was abnormal pre-intervention and improved or normalized after intervention in most cases: biphasic mitral inflow was present in 5/25 (20%) cases pre-intervention and in 21/23 (91%) post-intervention (P < 0.001); moderate or severe mitral regurgitation was present in 14/26 (54%) cases pre-intervention and in 5/23 (22%) post-intervention (P = 0.02); bidirectional flow across the patent foramen ovale was present in 0/26 cases pre-intervention and in 6/25 (24%) post-intervention (P = 0.01); antegrade flow in the transverse arch was present in 0/25 cases pre-intervention and in 17/26 (65%) post-intervention (P < 0.001). The left ventricular ejection fraction increased from 19 +/- 10% pre-intervention to 39 +/- 14% post-intervention (P < 0.001). These changes were not observed in control fetuses (n = 18).Fetal aortic valvuloplasty, when technically successful, improves left ventricular systolic function and left heart Doppler characteristics.

    View details for DOI 10.1002/uog.5132

    View details for Web of Science ID 000250732900006

    View details for PubMedID 17764106

  • Pathophysiology, management, and outcomes of fetal hemodynamic instability during prenatal cardiac intervention PEDIATRIC RESEARCH Mizrahi-Arnaud, A., Tworetzky, W., Bulich, L. A., Wilkins-Haug, L. E., Marshall, A. C., Benson, C. B., Lock, J. E., McElhinney, D. B. 2007; 62 (3): 325-330

    Abstract

    Prenatal cardiac intervention (PCI) may favorably alter the in utero course of some congenital heart defects. In our preliminary experience with PCI, fetal hemodynamic instability (FHI) characterized by bradycardia and ventricular dysfunction was common. This study evaluated the pathophysiology, management, and short-term outcomes of FHI during PCI for aortic stenosis with evolving hypoplastic left heart syndrome (HLHS), HLHS with restrictive atrial septum, pulmonary atresia with intact ventricular septum, and hydrops due to structural heart disease. From 2000 to 2006, 83 fetuses underwent PCI, with ventricular access in 63, atrial access in 17, and both in three. FHI occurred in 37 fetuses (45%). FHI was associated with transventricular PCI (all but one case of FHI; p < 0.001) and large hemopericardium (n=9; p=0.07). Prolonged FHI was associated with severe ventricular distortion during ventricular puncture (p = 0.06). FHI was treated with resuscitation medications in 31 of 37 fetuses and resolved in all 37. Five fetuses died within 1 d of PCI: four had FHI and one had a massive hemopericardium. FHI is common and clinically important during transventricular PCI and may be caused by a ventricular reflex or reduced cardiac output from cardiac distortion during ventricular puncture. Hemopericardium may be causative in a subset of fetuses.

    View details for Web of Science ID 000248973200018

    View details for PubMedID 17622948

  • Outcome after Fontan failure and takedown to an intermediate palliative circulation ANNALS OF THORACIC SURGERY Almond, C. S., Mayer, J. E., Thiagarajan, R. R., Blume, E. D., del Nido, P. J., McElhinney, D. B. 2007; 84 (3): 880-887

    Abstract

    Fontan takedown to an intermediate palliative circulation is an important treatment option for patients with acute or subacute failure of a Fontan circulation from a variety of causes. Little is known about the subsequent outcome of these patients or their potential candidacy for a second attempt at Fontan completion.Patients followed up at Children's Hospital Boston who underwent takedown of a Fontan circulation to an intermediate palliative circulation within 1 year of Fontan completion were reviewed.Between 1979 and 2006, 53 patients underwent Fontan takedown at a median age of 2.3 years (range, 0.3 to 36.5 years). Takedown was performed during the Fontan procedure itself in 12 patients (22%), within the first postoperative month in 31(58%), and between 1 month and 1 year in 10 (18%). Overall, 29 patients (55%) survived the early period after takedown, and 19 ultimately underwent successful Fontan completion a median of 4.6 years after takedown; all but one was alive a median of 6.4 years later. Thirteen (68%) of the 19 had treatable abnormalities contributing to Fontan failure.Fontan takedown can provide effective stabilization of the acutely or subacutely failing Fontan circulation, although a substantial number of patients die early despite Fontan takedown. Subjects surviving the perioperative period can often undergo uneventful redo Fontan. A thorough evaluation for treatable abnormalities should be performed in all patients with a failing Fontan circulation and in patients who undergo Fontan takedown.

    View details for DOI 10.1016/j.athoracsur.2007.02.092

    View details for Web of Science ID 000248982400025

    View details for PubMedID 17720394

  • Acute and intermediate outcomes, and evaluation of injury to the aortic wall, as based on 15 years experience of implanting stents to treat aortic coarctation CARDIOLOGY IN THE YOUNG Qureshi, A. M., McElhinney, D. B., Lock, J. E., Landzberg, M. J., Lang, P., Marshall, A. C. 2007; 17 (3): 307-318

    Abstract

    Stenting for aortic coarctation has been shown to be effective in the short term. The safety and longer term efficacy of transcatheter therapy, however, must be well established if the technique is to be widely accepted as an alternative to surgery. In order to determine the frequency, spectrum, and outcome of injury to the aortic wall caused by angioplasty or stenting of aortic coarctation, the nomenclature of mural injury in these patients must be adapted to the conditions of transcatheter therapy.Between 1989 and July 2005, we inserted stents in 153 patients with aortic coarctation, their median age being 15.8 years. Prior aortic interventions had been performed in 98 patients, and preexisting aneurysms were observed in 19. Stenting resulted in a significant reduction of the gradient across the site of coarctation, from a median of 30 millimetres of mercury to zero (p less than 0.001), with a residual gradient within the aortic arch of 20 millimetres of mercury or more in 5% of patients. Acute injuries to the aortic wall, other than therapeutic tears, were observed in 3 patients (2%), none of whom required surgery. At median follow-up of 2.5 years, this being more than 5 years in 30 patients, 4 patients had died, albeit none from complications relating to stenting or catheterization. Acute injuries to the aortic wall did not progress, and new aneurysms were observed in 6% of patients subsequent to follow-up imaging. Stent fractures, and jailed or partially covered brachiocephalic vessels, were observed in 12, and 49, patients, respectively, but did not result in haemodynamic or embolic complications.Stenting for aortic coarctation results in consistent relief of the gradient, and few serious complications in the short and intermediate term. Serious injuries to the aortic wall are uncommon in our experience, and can be minimized with a focus on technical measures, such as pre-dilation before stenting.

    View details for DOI 10.1017/S1047951107000339

    View details for Web of Science ID 000247220000011

    View details for PubMedID 17319978

  • Fetal tricuspid valve size and growth as predictors of outcome in pulmonary atresia with intact ventricular septum PEDIATRICS Salvin, J. W., McElhinney, D. B., Colan, S. D., Gauvreau, K., del Nido, P. J., Jenkins, K. J., Lock, J. E., Tworetzky, W. 2006; 118 (2): E415-E420

    Abstract

    Pulmonary atresia with intact ventricular septum is a complex congenital cardiovascular anomaly that frequently requires single ventricle palliation. Fetal diagnosis of pulmonary atresia with intact ventricular septum is common, but the natural history of pulmonary atresia with intact ventricular septum diagnosed in midgestation, predictors of neonatal anatomy, and predictors of biventricular repair have not been determined. The objective of this study was to determine whether the size and rate of growth of the fetal tricuspid valve predict neonatal anatomy and biventricular repair.Twenty-three fetuses diagnosed with pulmonary atresia with intact ventricular septum between 1990 and 2004 were studied. Of 13 fetuses with a midgestation fetal tricuspid valve z score < or = -3, 1 achieved biventricular repair, compared with 5 of 5 with a tricuspid valve z score > -3. Of 13 fetuses with a midgestation fetal tricuspid valve z score < or = -3, 8 were diagnosed postnatally with a right ventricular dependent coronary circulation, compared with none with a tricuspid valve z score > -3. Midgestation and late gestation fetal tricuspid valve z scores correlated with neonatal tricuspid valve z score. The average rate of tricuspid valve growth between mid- and late fetal echocardiograms was significantly lower in patients who did not achieve biventricular repair than in those who did (0.012 +/- 0.008 cm per week vs 0.028 +/- 0.014 cm per week).Fetal tricuspid valve z score and rate of growth predict postnatal outcome in pulmonary atresia with intact ventricular septum. These findings may have important implications for prenatal counseling and selection of patients for fetal pulmonary valve dilation.

    View details for DOI 10.1542/peds.2006-0428

    View details for Web of Science ID 000239440600099

    View details for PubMedID 16882782

  • Validation and re-evaluation of a discriminant model predicting anatomic suitability for biventricular repair in neonates with aortic stenosis JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Colan, S. D., McElhinney, D. B., Crawford, E. C., Keane, J. F., Lock, J. E. 2006; 47 (9): 1858-1865

    Abstract

    The purpose of this study was to validate and re-evaluate our previously reported scoring systems for predicting optimal management in neonates with aortic stenosis (AS).In 1991, we reported a multivariate discriminant equation and an ordinal scoring system for predicting which neonates with AS are suitable for biventricular repair and which are better served by single ventricle management.Retrospective analysis was performed to: 1) validate our scoring systems in 89 additional neonates with AS and normal mitral valve area, 2) assess the effects of 5% measurement variation on predictive scores, 3) evaluate our cohort with the Congenital Heart Surgeons' Society scoring system, and 4) repeat the discriminant analysis on the basis of all 126 patients.The original scores each predicted outcome accurately in 68 patients (77%). Minor (5%) measurement variation changed the outcome predicted by the discriminant equation in 8 patients (9%) and by the threshold system in 13 patients (15%). The most accurate model for predicting survival with a biventricular circulation among the full cohort is: 10.98 (body surface area) + 0.56 (aortic annulus z-score) + 5.89 (left ventricular to heart long-axis ratio) - 0.79 (grade 2 or 3 endocardial fibroelastosis) - 6.78. With a cutoff of -0.65, outcome was predicted accurately in 90% of patients.Both of our original scoring systems are less accurate at predicting outcome than in our original analysis. Revised discriminant analysis yielded a model similar to our original equation that was 90% accurate at predicting survival with a biventricular circulation among neonates with AS and a mitral valve area z-score >-2.

    View details for DOI 10.1016/j.jacc.2006.02.020

    View details for Web of Science ID 000237235500016

    View details for PubMedID 16682313

  • Fetal aortic valve stenosis and the evolution of hypoplastic left heart syndrome - Patient selection for fetal intervention CIRCULATION Makikallio, K., McElhinney, D. B., Levine, J. C., Marx, G. R., Colan, S. D., Marshall, A. C., Lock, J. E., Marcus, E. N., Tworetzky, W. 2006; 113 (11): 1401-1405

    Abstract

    Fetal aortic valvuloplasty may prevent progression of aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS). Predicting which fetuses with AS will develop HLHS is essential to optimize patient selection for fetal intervention. The aim of this study was to define echocardiographic features associated with progression of midgestation fetal AS to HLHS.Fetal echocardiograms were reviewed from 43 fetuses diagnosed with AS and normal left ventricular (LV) length at < or =30 weeks' gestation. Of 23 live-born patients with available follow-up data, 17 had HLHS and 6 had a biventricular circulation. At the time of diagnosis, LV length, mitral valve, aortic valve, and ascending aortic diameter Z-scores did not differ between fetuses that ultimately developed HLHS and those that maintained a biventricular circulation postnatally. However, all of the fetuses that progressed to HLHS had retrograde flow in the transverse aortic arch (TAA), 88% had left-to-right flow across the foramen ovale, 91% had monophasic mitral inflow, and 94% had significant LV dysfunction. In contrast, all 6 fetuses with a biventricular circulation postnatally had antegrade flow in the TAA, biphasic mitral inflow, and normal LV function. With advancing gestation, growth arrest of left heart structures became evident in fetuses developing HLHS.In midgestation fetuses with AS and normal LV length, reversed flow in the TAA and foramen ovale, monophasic mitral inflow, and LV dysfunction are predictive of progression to HLHS. These physiological features may help refine patient selection for fetal intervention to prevent the progression of AS to HLHS.

    View details for DOI 10.1161/CIRCULATIONAHA.105.588194

    View details for Web of Science ID 000236131700008

    View details for PubMedID 16534003

  • Incorporation of the hepatic veins into the cavopulmonary circulation in patients with heterotaxy and pulmonary arteriovenous malformations after a Kawashima procedure ANNALS OF THORACIC SURGERY McElhinney, D. B., Kreutzer, J., Lang, P., Mayer, J. E., Del Nido, P. J., Lock, J. E. 2005; 80 (5): 1597-1603

    Abstract

    In patients with polysplenia syndrome and azygous continuation of an interrupted inferior vena cava (IVC), pulmonary arteriovenous malformations (PAVMs) are relatively common after bidirectional cavopulmonary anastomosis (BCPA, Kawashima procedure). Resolution of PAVMs after hepatic vein (HV) inclusion into the cavopulmonary circulation has been reported, but there has been no systematic investigation of the effects of this therapy in a population of more than 3 patients.We studied 16 patients with heterotaxy, univentricular congenital heart disease, and azygous continuation of the IVC who underwent incorporation of the HV into the cavopulmonary circuit for treatment of significant PAVMs after a Kawashima procedure.The median preoperative systemic arterial oxygen saturation (SsaO2) was 76% (65%-85%), compared with 89% (85% to 92%) early after BCPA. Among 15 early survivors, the median early postoperative SsaO2 was 76% (56%-85%). In 11 of the 15 survivors, SsaO2 rose to 90% or greater within a year and remained at 93% or greater at follow-up of 2.8 to 10 years. Four patients had persistent hypoxemia and residual PAVMs at follow-up catheterization 1.5 to 8 years postoperatively; these patients had the most severe hypoxemia prior to HV inclusion, and in 2 the residual PAVMs were unilateral, with HV flow streaming to the contralateral lung, in which PAVMs had resolved.Hypoxemia resolved after cavopulmonary incorporation of the HV in the majority of our patients with PAVMs after the Kawashima operation, presumably due to a combination of PAVM resolution and elimination of hepatic venoatrial right-to-left shunting. These findings support the theory that development of PAVMs is facilitated by exclusion of HV effluent from the pulmonary circulation.

    View details for DOI 10.1016/j.athoracsur.2005.05.101

    View details for Web of Science ID 000232970500005

    View details for PubMedID 16242423

  • Creation of a brachial arteriovenous fistula for treatment of pulmonary arteriovenous malformations after cavopulmonary anastomosis ANNALS OF THORACIC SURGERY McElhinney, D. B., Marshall, A. C., Lang, P., Lock, J. E., Mayer, J. E. 2005; 80 (5): 1604-1609

    Abstract

    Pulmonary arteriovenous malformations (PAVMs) occur in approximately 20% of patients after unidirectional superior cavopulmonary anastomosis (CPA), and frequently after bidirectional CPA in patients with polysplenia syndrome. It is hypothesized that exclusion of a growth-modulating factor produced in the liver may predispose to PAVM formation. Resolution of PAVMs after inclusion of hepatic venous effluent into the cavopulmonary circulation has been reported. An upper extremity systemic arteriovenous (AV) fistula may be created to augment pulmonary blood flow and improve oxygenation in hypoxemic patients with CPA, but there has been no systematic investigation of the effects of such fistulas on PAVMs after CPA.We studied 11 patients with PAVMs who underwent creation of a brachial AV fistula a median of 11 years after CPA.Eight patients had discontinuous pulmonary arteries or unilateral flow of a bidirectional CPA and were not considered good candidates for Fontan completion; the other 3 patients had polysplenia and unilateral hepatic venous streaming after Fontan completion. Three patients died of progressive complications of their heart disease 4 to 18 months after AV fistula creation. Pulmonary arteriovenous malformations resolved after creation of a brachial AV fistula in 4 of 5 surviving patients with unilateral flow of a superior CPA, but in none of 3 patients with polysplenia who had unilateral hepatic venous streaming after Fontan completion and PAVMs in the contralateral lung.These findings are consistent with the "hepatic factor" hypothesis, according to which the development of PAVMs is facilitated when an unidentified factor produced or metabolized in the liver does not reach the pulmonary circulation before traversing another capillary bed. Patients with unilateral superior CPA flow and PAVMs who are not considered candidates for Fontan completion may benefit from a brachial AV fistula.

    View details for DOI 10.1016/j.athoracsur.2005.05.100

    View details for Web of Science ID 000232970500006

    View details for PubMedID 16242424

  • Improving outcomes in fetuses and neonates with congenital displacement (Ebstein's malformation) or dysplasia of the tricuspid valve AMERICAN JOURNAL OF CARDIOLOGY McElhinney, D. B., Salvin, J. W., Colan, S. D., Thiagarajan, R., Crawford, E. C., Marcus, E. N., Del Nido, P. J., Tworetzky, W. 2005; 96 (4): 582-586

    Abstract

    From 1984 to 2004, 66 patients were diagnosed with Ebstein's malformation (n = 61) or congenital tricuspid valve (TV) dysplasia (n = 5) in utero or during the first month of life. Of these, 33 were diagnosed by fetal echocardiography at a median gestational age of 22 weeks, and 33 were diagnosed postnatally at a median age of 1 day (range 1 to 27). In 8 of the 33 prenatally diagnosed patients (24%), the pregnancies were terminated; in 9 (27%), the fetuses died in utero, and in 16 (49%), the fetuses survived to birth. Seven prenatally diagnosed patients survived beyond the neonatal period (21% of 33). Of the 49 neonates, 35 (71%) survived to hospital discharge and beyond 1 month of age. Independent predictors of death by multivariable logistic regression analysis included right atrial area index >1, the absence of anterograde flow across the pulmonary valve, and diagnosis before 1997. Although outcomes in fetuses and neonates with congenital anomalies of the TV have improved in more recent experience, survival in patients at the severe end of the spectrum remains poor. To improve outcomes in this group of high-risk patients, novel approaches to management may be indicated.

    View details for DOI 10.1016/j.amjcard.2005.04.024

    View details for Web of Science ID 000231255100022

    View details for PubMedID 16098316

  • Left heart growth, function, and reintervention after balloon aortic valvuloplasty for neonatal aortic stenosis CIRCULATION McElhinney, D. B., Lock, J. E., Keane, J. F., Moran, A. M., Colan, S. D. 2005; 111 (4): 451-458

    Abstract

    Transcatheter balloon aortic valvuloplasty (BAVP) has become the first-line treatment for critical aortic stenosis (AS) in neonates. However, little is known about the growth and function of left heart structures or about patterns of reintervention on the left heart after neonatal BAVP.Between 1985 and 2002, 113 patients underwent neonatal BAVP at < or =60 days of age. There were 16 early deaths (14%), with a significant decrease from 1985 to 1993 (22%) to 1994 to 2002 (4%), and 6 patients had successful early conversion to a univentricular circulation. In the short term, the mean relative gradient reduction was 54+/-26%, and significant aortic regurgitation (AR) developed in 15% of patients. The 91 early survivors with a biventricular circulation were followed up for 6.3+/-5.3 years, during which time there was a steady increase in the frequency of significant AR. Freedom from moderate or severe AR was 65% at 5 years. In almost all patients with a baseline aortic annulus z score less than -1, the annulus diameter increased to within the normal range within 1 to 2 years. Similarly, left ventricular (LV) end-diastolic dimension z scores, which ranged from -5 to 7.5 before BAVP, normalized within 1 to 2 years in nearly all patients with a predilation z score less than -1. Among early survivors with a biventricular circulation, reintervention-free survival on the LV outflow tract was 65% at 1 year and 48% at 5 years, with younger age, higher pre- and post-BAVP gradients, and a larger balloon-annulus diameter ratio associated with decreased reintervention-free survival (P<0.01). Seventeen surgical interventions were performed on the aortic valve in 15 patients, including replacement in 7. Survival free from aortic valve replacement was 84% at 5 years.BAVP for AS during the first 60 days of life results in short-term relief of AS in the majority of patients. Among early survivors, initially small left heart structures may be associated with worse subacute outcomes but typically normalize within 1 year. Reintervention for residual/recurrent AS or iatrogenic AR is relatively common, particularly during the first year after BAVP, but aortic valve replacement during early childhood is seldom necessary.

    View details for DOI 10.1161/01.CIR.0000153809.88286.2E

    View details for Web of Science ID 000226692600014

    View details for PubMedID 15687133

  • Recombinant human growth hormone treatment for dilated cardiomyopathy in children PEDIATRICS McElhinney, D. B., Colan, S. D., Moran, A. M., Wypij, D., Lin, M., Majzoub, J. A., Crawford, E. C., Bartlett, J. M., McGrath, E. A., Newburger, J. W. 2004; 114 (4): E452-E458

    Abstract

    Dilated cardiomyopathy (DCM) is one of the most common causes of heart failure among children and is often progressive despite maximal medical therapy. Heart failure is characterized by a number of neurohormonal abnormalities, including derangements in the growth hormone (GH)/insulin-like growth factor-1 (IGF-1) signaling axis. Decreased serum levels of GH, which acts on cardiac myocytes primarily through IGF-1, are associated with impaired myocardial growth and function, which can be improved with restoration of GH/IGF-1 homeostasis. In animal models and among human adults with heart failure attributable to DCM, treatment with GH results in acquisition of left ventricular (LV) mass and improved LV function, through a combination of mechanisms. We undertook this study to determine the effects of recombinant human GH on LV function and mass among children with stable LV dysfunction attributable to DCM.We performed a prospective, single-center, randomized, partially blinded, crossover trial among children 1 to 19 years of age with DCM and cardiac dysfunction of > or =6-month duration. After enrollment, patients were randomly assigned to receive treatment for 6 months with either conventional therapy (determined by the patient's primary cardiologist) plus recombinant human GH (0.025-0.04 mg/kg per day), administered as daily subcutaneous injections, or conventional therapy alone. Patients were then crossed over to the other treatment strategy for 6 months. The primary outcome measure was change in LV shortening fraction (SF). Other echocardiographic indices of LV function, somatic growth, and somatotropic/thyroid hormone levels were also monitored.Only 8 of an intended 15 patients were enrolled, because of a combination of factors. Two patients withdrew during the study as a result of declining LV function requiring transplantation. LV SF did not change significantly during GH treatment, although both LV SF and LV SF z score were higher 6 months after cessation of GH treatment than at baseline. LV ejection fraction increased during GH therapy to a degree that approached significance. Height and weight percentiles for age increased significantly during GH therapy and remained higher 6 months after treatment. Annualized height velocity during GH treatment (13.7 +/- 3.3 cm/year, >97th percentile for all patients) was significantly higher than that after GH discontinuation (3.2 +/- 3.5 cm/year). Serum levels of IGF-1 and IGF-binding protein-3 were significantly higher after 6 months of GH treatment and 6 months after discontinuation of GH treatment than at baseline. There were no adverse events related to GH treatment.In this prospective, single-center, randomized, partially blinded, crossover trial, recombinant human GH was administered to 8 pediatric patients with stable chronic heart failure secondary to DCM. Because of unanticipated difficulty enrolling eligible patients, the study was underpowered to detect changes in our primary outcome measure of the magnitude we projected. Nevertheless, we did observe several notable cardiovascular effects of GH treatment, including a trend toward improved LV ejection fraction during the course of GH treatment and significantly improved LV SF, SF z score, and LV end systolic stress z score 6 months after discontinuation of GH treatment (relative to baseline values). Given the fact that levels of IGF-1, the primary myocardial effector of GH signaling, remained significantly higher 6 months after GH treatment than at baseline, the improvement in LV functional indices 6 months after discontinuation of therapy may represent progression or perpetuation of a GH treatment effect. In addition to its cardiovascular effects, GH therapy was associated with significant acceleration of somatic growth. The benefits of GH were not associated with significant attributable side effects, although 2 patients developed progressive LV dysfunction during the study and underwent cardiac transplantation.

    View details for DOI 10.1542/peds.2004-0072

    View details for Web of Science ID 000224242200009

    View details for PubMedID 15466071

  • Transcatheter device closure of congenital and postoperative residual ventricular septal defects CIRCULATION Knauth, A. L., Lock, J. E., Perry, S. B., McElhinney, D. B., Gauvreau, K., Landzberg, M. J., Rome, J. J., Hellenbrand, W. E., Ruiz, C. E., Jenkins, K. J. 2004; 110 (5): 501-507

    Abstract

    Our purpose was to describe a 13-year experience with patients undergoing transcatheter device closure of unrepaired congenital or postoperative residual ventricular septal defects (VSDs).Since 1989, 170 patients (median age, 3.9 years) have undergone catheterization for closure of 1 or more congenital (n=92) or postoperative (n=78) residual VSDs using successive generations of STARFlex-type devices. Outcomes included echocardiographic assessment of residual flow and device position, assessment of VSD shunt/severity, and adverse events. Among 168 patients in whom device implantation was performed, between 1 and 7 devices were placed per patient (median, 1), with multiple devices placed in 40%. There was a significant decrease in left-to-right shunting after device implantation (P<0.001) and significant improvement in VSD size/severity, and device position proved stable. Of 332 adverse events, 39 were related to the device and 261 were related to the catheterization; all but 5 occurred in the periprocedural period. At a median follow-up of 24 months (0 to 154 months), 14 patients had died and 18 had device(s) explanted.Congenital and postoperative VSD closure using STARFlex-type devices resulted in stable improvement in clinical status and decreased interventricular shunting. Although periprocedural events occurred frequently, late events caused by the device were rare. Transcatheter device closure is an effective management option for patients with complex muscular VSDs that are difficult to approach surgically and for postoperative residual VSDs.

    View details for DOI 10.1161/01.CIR.0000137116.12176.A6

    View details for Web of Science ID 000223055600006

    View details for PubMedID 15262841

  • Hypoplastic left heart syndrome with intact or highly restrictive atrial Septum - Outcome after neonatal transcatheter atrial septostomy CIRCULATION Vlahos, A. P., Lock, J. E., McElhinney, D. B., van der Velde, M. E. 2004; 109 (19): 2326-2330

    Abstract

    Hypoplastic left heart syndrome (HLHS) with intact or very restrictive atrial septum is a highly lethal combination. We review our 13-year institutional experience treating this high-risk subgroup of patients with emergent catheter therapy.Infants with HLHS requiring catheter septostomy within the first 2 days of life were compared with a matched control group with adequate interatrial communication. Preoperative, early postoperative, and medium-term survival were evaluated. Earlier experience was compared with recent results to assess the effect of changes in catheterization and surgical and intensive care unit management strategies over the study period. From 1990 to 2002, 33 newborns with HLHS (11% of newborns with HLHS managed during this period) underwent urgent/semiurgent catheterization to create or enlarge an interatrial communication before surgical palliation. Preoperative and early postoperative mortality were high (48%) compared with control HLHS patients, regardless of prenatal diagnosis and despite successful catheter-based atrial septostomy with clinical stabilization. Mortality trended down during the later part of the study period. Those who survived the neonatal period had late survival, pulmonary artery pressure, and resistance similar to those of control subjects.Neonatal mortality in the subgroup of HLHS patients with intact or highly restrictive atrial septum remains high despite successful urgent septostomy. Persistently poor outcomes for these patients have prompted efforts at our center to develop techniques for fetal intervention for this condition, in the hope that prenatal relief of left atrial and pulmonary venous hypertension may promote normal pulmonary vascular and parenchymal development and improve both short- and long-term outcomes.

    View details for DOI 10.1161/01.CIR.0000128690.35860.C5

    View details for Web of Science ID 000221477800014

    View details for PubMedID 15136496

  • Association between cardiac tumors and tuberous sclerosis in the fetus and neonate AMERICAN JOURNAL OF CARDIOLOGY Tworetzky, W., McElhinney, D. B., Margossian, R., Moon-Grady, A. J., Sallee, D., Goldmuntz, E., van der Velde, M. E., Silverman, N. H., Allan, L. D. 2003; 92 (4): 487-489

    Abstract

    A retrospective review was performed in 94 patients with > or =1 cardiac tumors seen on prenatal or neonatal echocardiography at 5 major referral centers. Tuberous sclerosis was present in 68 patients diagnosed with a cardiac tumor in utero or during the neonatal period, including 61 of 64 with multiple tumors.

    View details for DOI 10.1016/S0002-9149(03)00677-5

    View details for Web of Science ID 000184650200029

    View details for PubMedID 12914889

  • Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome 47th Annual Scientific Session of the American-College-of-Cardiology Tworetzky, W., McElhinney, D. B., Reddy, V. M., Brook, M. M., Hanley, F. L., Silverman, N. H. LIPPINCOTT WILLIAMS & WILKINS. 2001: 1269–73

    Abstract

    Hypoplastic left heart syndrome (HLHS) is frequently diagnosed prenatally, but this has not been shown to improve surgical outcome.We reviewed patients with HLHS between July 1992 and March 1999 to determine the influence of prenatal diagnosis on preoperative clinical status, outcomes of stage 1 surgery, and parental decisions regarding care. Of 88 patients, 33 were diagnosed prenatally and 55 after birth. Of 33 prenatally diagnosed patients, 22 were live-born, and pregnancy was terminated in 11. Of 22 prenatally diagnosed patients who were live-born, 14 underwent surgery, and parents elected to forego treatment in 8. Of 55 patients diagnosed postnatally, 38 underwent surgery, and 17 did not because of parental decisions or clinical considerations. Prenatally diagnosed patients were less likely to undergo surgery than patients diagnosed after birth (P:=0.008). Among live-born infants, there was a similar rate of nonintervention. Among patients who underwent surgery, survival was 75% (39/52). All patients who had a prenatal diagnosis and underwent surgery survived, whereas only 25 of 38 postnatally diagnosed patients survived (P:=0.009). Patients diagnosed prenatally had a lower incidence of preoperative acidosis (P:=0.02), tricuspid regurgitation (P:=0.001), and ventricular dysfunction (P:=0.004). They were also less likely to need preoperative inotropic medications or bicarbonate (P:=0.005). Preoperative factors correlating with early mortality included postnatal diagnosis (P:=0.009), more severe acidosis (P:=0.03), need for bicarbonate or inotropes (P:=0.008 and 0.04), and ventricular dysfunction (P:=0.05).Prenatal diagnosis of HLHS was associated with improved preoperative clinical status and with improved survival after first-stage palliation in comparison with patients diagnosed after birth.

    View details for Web of Science ID 000167562200017

    View details for PubMedID 11238272

  • Transesophageal echocardiographic predictors for successful transcatheter closure of defects within the oval fossa using the CardioSEAL((R)) septal occlusion device CARDIOLOGY IN THE YOUNG Momenah, T. S., McElhinney, D. B., Brook, M. M., Moore, P., Silverman, N. H. 2000; 10 (5): 510-518

    Abstract

    To define the utility of transesophageal echocardiography in predicting the likelihood of a successful procedure and residual shunting in patients undergoing transcatheter closure of defects within the oval fossa using the CardioSEAL device.Transesophageal echocardiography is used to monitor transcatheter closure of interatrial defects within the the oval fossa, but predictors of successful closure and residual shunting have yet to be determined.We reviewed transesophageal echocardiograms obtained from 26 consecutive patients undergoing attempted transcatheter closure of interatrial defects within the oval fossa between January, 1997 and May, 1998. Assessment of the atrial septum, the septal defect, and the rims of the oval fossa bordering the defect was performed in 3 planes: longitudinal, 4-chamber, and basal short-axis.Closure proved successful in 24 patients (92%). The defect was significantly larger, and the anterosuperior rim of the defect smaller, in the 2 patients in whom occlusion was not successful. Residual shunting 24 hrs after closure was detected in 14 patients. Significant predictors of leakage included smaller posterior and superior rims, a larger shunt prior to closure, and herniation of a one left atrial arm of the device into the right atrium. In all cases, the sites of leakage were the superior rim of the defect at the superior cavo-atrial junction, and the anterosuperior rim behind the aortic root. Herniation of a left atrial arm into the right atrium was seen in 7 patients (29%). In all, it was the anterosuperior arm which herniated Doppler color flow was suboptimal in detecting residual leaks, and was enhanced substantially with the use of contrast echocardiography.Transesophageal echocardiography allows excellent assessment of the oval fossa and deficiencies of its floor in all of their dimensions. It is an important tool for guiding the deployment of the occlusion device in patients undergoing attempted transcatheter closure of defects within the fossa. Contrast echocardiography should be used for optimal detection of residual shunting.

    View details for Web of Science ID 000089640900013

    View details for PubMedID 11049127

  • Incidence and implications of systemic to pulmonary collaterals after bidirectional cavopulmonary anastomosis ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Tworetzky, W., Petrossian, E., Hanley, F. L., Moore, P. 2000; 69 (4): 1222-1228

    Abstract

    Systemic to pulmonary arterial collaterals often develop after bidirectional cavopulmonary anastomosis (BCPA). It has been proposed that such collaterals may be related to perioperative outcome and duration of effusions after the modified Fontan procedure. However, the incidence and significance of collaterals after BCPA remain uncertain.To evaluate risk factors for, and significance of, such collaterals, we reviewed angiographic and clinical data for all 76 patients who underwent BCPA between January 1990 and June 1996 and had follow-up catheterization during or before 1997.The median age at BCPA was 10 months, and the median duration from BCPA to follow-up catheterization was 18 months. Arterial collaterals were detected on follow-up catheterization in 45 patients (59%). Factors associated with collateral development included a prior right-sided systemic-to-pulmonary arterial shunt, lower pre-BCPA end-diastolic ventricular pressure and pulmonary vascular resistance, and use and duration of cardiopulmonary bypass during the BCPA operation. Fourteen of the 45 patients (30%) underwent coil embolization of the collaterals. Forty-three patients have undergone extracardiac conduit Fontan, with 1 early and 1 late death. Collaterals were present in 22 of these patients, 7 of whom underwent pre-Fontan embolization. The duration from BCPA to Fontan was longer in patients with collaterals, but these patients were not more likely to have prolonged effusions than those without, and the duration of tube thoracostomy was significantly shorter in patients with collaterals. Embolization of collaterals did not affect the duration of effusions.Systemic-to-pulmonary arterial collaterals are common after BCPA. In contrast to prior reports, collaterals were not associated with a higher incidence of prolonged effusions after the Fontan procedure in our experience, and did not correlate with poor outcome.

    View details for Web of Science ID 000086808500059

    View details for PubMedID 10800823

  • Issues and outcomes in the management of supravalvar aortic stenosis ANNALS OF THORACIC SURGERY McElhinney, D. B., Petrossian, E., Tworetzky, W., Silverman, N. H., Hanley, F. L. 2000; 69 (2): 562-567

    Abstract

    Supravalvar stenosis of the aorta is an uncommon congenital cardiac anomaly that involves not only the supravalvar aorta but the entire aortic root. Despite considerable attention to the importance of maintaining the integrity of the aortic root during supravalvar reconstruction, there has been little focus on the management of other components of the aortic root and left ventricular outflow tract, including the aortic valve, subvalvar region, and coronary arteries.We reviewed the records of 36 consecutive patients with supravalvar aortic stenosis who underwent repair from 1992-1998 (median age, 4 years). Discrete stenosis was present in 29 patients, whereas the remaining 7 had the diffuse form of the disease. Associated anomalies of the aortic root and adjacent structures were present in 23 patients. The median pressure gradient across the left ventricular outflow tract was 70 mm Hg. Supravalvar stenosis was relieved by extended aortoplasty with a Y-shaped patch in 18 patients, resection of the stenotic segment of ascending aorta at the sinotubular junction with end-to-end anastomosis of the ascending aorta in 7, the Ross procedure in 4, and other techniques in 7. Additional procedures included aortic valvuloplasty in 10 patients, resection of subvalvar stenosis in 11, and procedures on the coronary arteries in 2.There was 1 perioperative death, and no reoperations or other significant complications. During follow-up (median 33 months), there were no deaths and 3 reoperations for replacement of the aortic valve with a pulmonary autograft (n = 1) or mechanical prosthesis (n = 2). The median pressure gradient across the left ventricular outflow tract was 10 mm Hg.In patients with supravalvar aortic stenosis, abnormalities of the aortic valve, subaortic region, and coronary arteries are frequently present as well. Management of these issues is as critical to the long-term outcome of these patients as reconstruction of the supravalvar aorta. Aggressive valvuloplasty may help decrease the incidence of late aortic valve replacement, whereas the Ross procedure may be a preferable approach in some patients with complex outflow tract obstruction.

    View details for Web of Science ID 000085382200061

    View details for PubMedID 10735699

  • Congenital obstructive lesions of the right aortic arch ANNALS OF THORACIC SURGERY McElhinney, D. B., Tworetzky, W., Hanley, F. L., Rudolph, A. M. 1999; 67 (4): 1194-1202

    Abstract

    In the setting of normal cardiac situs, a right-sided aortic arch is uncommon. When a right arch does occur, it is typically in conjunction with other congenital cardiovascular anomalies, especially defects with abnormal right ventricular outflow. Congenital obstruction of a right arch, caused by coarctation, interruption, or cervical arch, is extremely rare.We reviewed our experience and all reported cases of right aortic arch with coarctation of the aorta, interrupted arch, or obstruction of a cervical arch in the setting of normal cardiac situs and topology.Since 1992, 4 such patients have undergone repair at our institution, including 1 with interrupted arch, 1 with coarctation of a mirror image arch, and 2 with obstruction of a cervical arch. In addition to these 4 patients, 38 others have been described in the published reports: 15 with interrupted arch, 19 with coarctation, and 4 with obstruction of a cervical arch. Associated cardiac defects were uncommon, except for ventricular septal defect in patients with interrupted arch, but abnormalities of the brachiocephalic vessels were frequent. Except for most of the patients with interrupted right arch, the majority of patients described have undergone successful surgical repair.Although obstructive arch lesions are often grouped together, the etiologies of coarctation of the aorta, interrupted arch, and cervical arch with obstruction almost certainly differ. The rarity of such lesions among patients with right aortic arch may be explained in part by the fact that the fetal hemodynamic conditions associated with persistence of a right arch do not facilitate flow-related arch obstruction. In this review, we discuss these issues in detail, along with specific surgical considerations in the management of obstruction lesions of the right aortic arch.

    View details for Web of Science ID 000080115300078

    View details for PubMedID 10320289

  • Multiple ventricular septal defects: How and when should they be repaired? 78th Annual Meeting of the American-Association-for-Thoracic-Surgery Seddio, F., Reddy, V. M., McElhinney, D. B., Tworetzky, W., Silverman, N. H., Hanley, F. L. MOSBY-ELSEVIER. 1999: 134–39

    Abstract

    Congenital heart lesions with multiple ventricular septal defects remain a surgical challenge. Traditional approaches often rely on either ventriculotomy for exposure or palliation with pulmonary artery banding. However, indications for repair versus palliation and for various approaches to surgical exposure are not clearly defined.From July 1992 to January 1998, 45 patients with multiple (>/=2) ventricular septal defects (37 with associated lesions) underwent surgery. Median age was 86 days; all but 4 patients were infants. The mean number of defects was 3.7, and almost half of the patients had more than 3 defects. Apical muscular defects were present in 62% of patients. Thirty-one patients underwent primary complete repair through a right atriotomy or trans-semilunar valve approach (group 1), 8 had palliation (group 2), and 6 underwent complete repair after prior palliation elsewhere (group 3). No patient had a ventriculotomy.One early death occurred in a group 1 patient. Four patients who had had palliation (50%) underwent early reoperation for pulmonary artery band revision because of failure to thrive or band removal after spontaneous closure of the defects. At follow-up (median 22 months), there was 1 death in a group 2 patient (palliation) and 1 other group 2 patient required cardiac transplantation. The only late reoperation was for removal of the pulmonary artery band and closure of multiple apical defects in a group 2 (palliation) patient. No patients who underwent repair have hemodynamically significant residual defects.In our experience, palliation of multiple ventricular septal defects is associated with greater morbidity than primary repair. Multiple defects can almost always be repaired adequately in early infancy without ventriculotomy, although "Swiss-cheese" septum may be an indication for palliation.

    View details for Web of Science ID 000077898200027

    View details for PubMedID 9869767

  • Echocardiographic diagnosis alone for the complete repair of major congenital heart defects JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Tworetzky, W., McElhinney, D. B., Brook, M. M., Reddy, V. M., Hanley, F. L., Silverman, N. H. 1999; 33 (1): 228-233

    Abstract

    The study was done to determine the diagnostic accuracy of echocardiography alone in the preoperative diagnosis of children with major congenital heart defects undergoing primary complete repair.Although echocardiography is well established as the first-line imaging technique for the diagnosis of all forms of congenital heart disease, most institutions continue to perform cardiac catheterization prior to complete repair of more complex defects.To determine the diagnostic accuracy of echocardiography alone and echocardiography plus catheterization, we reviewed the records of 503 children with major congenital heart defects who underwent primary complete repair at our institution between July 1992 and June 1997. We included children with transposition of the great arteries, tetralogy of Fallot, double-chamber right ventricle, interrupted aortic arch, aortic coarctation, atrioventricular septal defect, truncus arteriosus, aortopulmonary septal defect, and totally anomalous pulmonary venous return. We excluded children with less complex defects such as isolated shunt lesions, as well as those with the most complex defects that would require surgical palliation (e.g., functional univentricular heart). We defined major errors as those that increased the surgical risk and minor errors as those that did not. Errors in diagnosis were determined at surgery.Eighty-two percent of children (412 of 503) underwent surgery after preoperative diagnosis by echocardiography alone. There were 9 major (2%) and 10 minor errors in the echocardiography alone group and 7 major and 5 minor errors in the echocardiography plus catheterization group. The most common type of error was misidentification of coronary artery anatomy in patients with transposition of the great arteries. No error in either group resulted in surgical morbidity or mortality.This study suggests that echocardiography alone is an accurate tool for the preoperative diagnosis of major congenital heart defects in most children undergoing primary complete repair, and may obviate the need for routine diagnostic catheterization.

    View details for Web of Science ID 000078088300036

    View details for PubMedID 9935035

  • Intramyocardial hematoma causing cardiac tamponade after repair of Ebstein malformation: Erroneous echocardiographic diagnosis as intracavitary thrombus JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Momenah, T. S., McElhinney, D. B., Brook, M. M., Teitel, D. F., Hanley, F. L., Silverman, N. H. 1998; 11 (11): 1087-1089

    Abstract

    Intramyocardial hematoma is an uncommon lesion, most often occurring after chest trauma, aortic valve disease, acute myocardial infarction, or coronary artery bypass surgery. We describe a 17-year-old patient who experienced cardiac tamponade after repair of Ebstein malformation. The malformation was caused by an enlarging intramyocardial hematoma that extended from the right atrium to the atrialized right ventricle. The hematoma was incorrectly diagnosed by echocardiography as an intracavitary thrombus, and the correct diagnosis was recognized only at the time of surgical intervention.

    View details for Web of Science ID 000077038900014

    View details for PubMedID 9812104

  • Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants < 6 months of age AMERICAN JOURNAL OF CARDIOLOGY McElhinney, D. B., Reddy, V. M., Tworetzky, W., Silverman, N. H., Hanley, F. L. 1998; 81 (2): 195-201

    Abstract

    The Richardson classification system for aortopulmonary septal defect (APSD) includes simple defects between the ascending aorta and pulmonary trunk (type I), defects extending distally to include the origin of the right main pulmonary artery (type II), and anomalous origin of the right main pulmonary artery from the ascending aorta with no other aortopulmonary communication (type III). These are rare lesions that must be repaired in early infancy to avoid development of pulmonary vascular disease. Few reports have focused on patients with complex, associated lesions who underwent repair in early infancy. Between 1972 and 1995, 24 patients with Richardson type I (n = 11), II (n = 7), or III (n = 6) defects underwent repair at ages ranging from 2 to 172 days (median 34). Twelve patients had complex, associated anomalies, including interrupted or hypoplastic arch (n = 9), tetralogy of Fallot with (n = 1) or without (n = 1) pulmonary atresia, and transposition of the great arteries (n = 1). The most recent 7 patients were diagnosed by echocardiography without cardiac catheterization. There were no early or late deaths among the 12 patients with simple APSD. Four patients with complex, associated lesions died in the early postoperative period and another died 4 months after surgery. All 6 surviving patients with interrupted arch have had recurrent obstruction at the arch repair site, although reintervention for this reason has been performed in only 2 patients. Altogether, 6 early survivors have required reintervention, and all survivors are in New York Heart Association class I at follow-up ranging from 2 to 25 years. Thus, long-term survival after repair of APSD in early infancy is excellent. Late sequelae are likely to be related either to associated lesions or to obstruction at the APSD repair site. Almost all cases of APSD in young infants can be diagnosed and evaluated by echocardiography without catheterization.

    View details for Web of Science ID 000071617500015

    View details for PubMedID 9591904

  • An institutional experience with the bidirectional cavopulmonary shunt: do we know enough about it? 22nd Annual Meeting of the Western-Thoracic-Surgical-Association Reddy, V. M., McElhinney, D. B., Moore, P., Bristow, J., Haas, G. S., Hanley, F. L. GREENWICH MEDICAL MEDIA LTD. 1997: 284–93