Bio

Clinical Focus


  • Pediatric Cardiology
  • Pediatrics, General

Academic Appointments


Professional Education


  • Residency:Children's Hospital of the King's Daughters (2000) VA
  • Internship:Children's Hospital of the King's Daughters (1999) VA
  • Medical Education:Eastern Virginia Medical School of Hampton Roads (1998) VA
  • Board Certification: Pediatric Cardiology, American Board of Pediatrics (2008)
  • Fellowship:Children's Hospital and Health Center- UC San Diego (2007) CA

Publications

Journal Articles


  • Surgical repair of anomalous aortic origin of a coronary artery. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery Mainwaring, R. D., Reddy, V. M., Reinhartz, O., Petrossian, E., Punn, R., Hanley, F. L. 2014

    Abstract

    Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart defect that has been associated with myocardial ischaemia and sudden death. There is an ongoing controversy over the indications for surgical intervention and the efficacy of that treatment compared with the natural history. The purpose of this study was to evaluate the medium-term results of surgical repair of AAOCA.Seventy-six patients underwent surgical repair of AAOCA at our institution from 1999 to 2013. There were 55 males and 21 females, and the median age at surgery was 15 years. Forty-seven (62%) of the 76 patients had an anomalous right coronary artery, 27 had an anomalous left coronary and 2 had an eccentric single coronary ostia. Forty-one patients had preoperative symptoms of myocardial ischaemia.Surgical repair was accomplished by unroofing of an intramural coronary in 55, reimplantation in 7 and pulmonary artery translocation in 14. There has been no early or late mortality, with a median duration of follow-up of 6 years. One patient presented with severe myocardial ischaemia and subsequently underwent heart transplantation a year following AAOCA surgery. The remaining patients have all remained free of cardiac symptoms.The results of this study demonstrate two major principles. First, surgical repair of AAOCA is quite safe in centres that take care of a significant number of patients with this entity. Secondly, the surgery is highly effective in eliminating symptoms of myocardial ischaemia. The growing amount of data on postoperative patients suggests that surgical repair can prevent the adverse events seen in the untreated 'natural' history. Based on these observations, it is our current recommendation that all teenagers identified with AAOCA should undergo surgical repair.

    View details for DOI 10.1093/ejcts/ezt614

    View details for PubMedID 24431169

  • Transcatheter Device Closure of a Congenital Aortic-Left Atrial Tunnel CONGENITAL HEART DISEASE Sun, H. Y., Buccola, K. J., Punn, R., Silverman, N. H., Peng, L. F., Perry, S. B., Balasubramanian, S. 2014; 9 (1): E23-E26

    Abstract

    Rare cases of aortic-left atrial tunnel exist in the literature. This case report highlights the echocardiographic characterization of this vascular anomaly and provides the first description of an aortic-left atrial tunnel closed by interventional cardiac catheterization in a pediatric patient.

    View details for DOI 10.1111/chd.12059

    View details for Web of Science ID 000329916300010

    View details for PubMedID 23601836

  • Ebstein anomaly and Trisomy 21: A rare association. Annals of pediatric cardiology Siehr, S. L., Punn, R., Priest, J. R., Lowenthal, A. 2014; 7 (1): 67-9

    Abstract

    This is a case report of a patient with Trisomy 21 with Ebstein anomaly, a ventricular septal defect, and acquired pulmonary vein stenosis; a rare combination, diagnosed during a routine neonatal examination.

    View details for DOI 10.4103/0974-2069.126569

    View details for PubMedID 24701093

  • Arterial Applanation Tonometry: Feasibility and Reproducibility in Children and Adolescents. American journal of hypertension Lowenthal, A., Evans, J. M., Punn, R., Nourse, S. E., Vu, C. N., Popat, R. A., Selamet Tierney, E. S. 2014

    Abstract

    Aortic pulse wave velocity (PWV) and augmentation index (AIx) are markers of vascular health and have recently been used in pediatric clinical trials. However, there are limited data on standardization of these measurements in pediatrics. The objective of this study was to prospectively test the feasibility and reproducibility of PWV and AIx in children and adolescents.We performed arterial tonometry on 2 different days within 2 weeks in 40 healthy subjects aged 10-19 years. PWV and AIx were measured in triplicate on each visit.The visits were separated by a mean of 3.08±3.7 days. We obtained PWV in 77 of 80 (96%) visits and AIx in 76 of 80 (95%) visits in triplicate. Intraclass correlation coefficients (ICCs) for PWV were 0.61 (95% confidence interval (CI) = 0-0.86) when at least 2 measurements and 0.92 (95% CI = 0-1) when 3 measurements were obtained at each visit that met the quality criteria established for adults by the manufacturer (n = 17 and 3 paired visits, respectively). For AIx, ICCs were 0.78 (95% CI = 0.58-0.88) and 0.81 (95% CI = 0.63-0.90) when measurements with an operator index ≥80, a measure of the quality of the waveform, were included (n = 39 and 36 paired visits, respectively).Arterial applanation tonometry is feasible and reproducible in healthy children and adolescents. AIx has excellent intervisit reproducibility, whereas the intervisit reproducibility of PWV relies on acquisition of multiple measurements that meet quality criteria established for adults. These results have implications for the methodology of future pediatric clinical trials in a population at increasingly higher risk for premature atherosclerosis.

    View details for DOI 10.1093/ajh/hpu034

    View details for PubMedID 24627445

  • Systemic hypertension in an infant with unrepaired tetralogy of Fallot: case report CARDIOLOGY IN THE YOUNG Khoury, M., Kallile, M., May, J., Punn, R. 2013; 23 (5): 746-748

    Abstract

    Patients with severe right ventricular outflow tract obstruction in tetralogy of Fallot typically have right-to-left shunting, resulting in low pulmonary blood flow and cyanosis. Here we present the case of an infant with tetralogy of Fallot and severe pulmonary valve stenosis, complicated by systemic hypertension, the presence of which altered flow dynamics and possibly prevented cyanosis.

    View details for DOI 10.1017/S1047951112001837

    View details for Web of Science ID 000323882900017

    View details for PubMedID 23149090

  • Echocardiographic Predictors of Early Postsurgical Myocardial Dysfunction in Pediatric Patients With Aortic Valve Insufficiency PEDIATRIC CARDIOLOGY Lowenthal, A., Tacy, T. A., Behzadian, F., Punn, R. 2013; 34 (6): 1335-1343

    Abstract

    In chronic aortic insufficiency (AI), left-ventricular (LV) dysfunction must be detected early to allow timely surgery. Strain and strain rate have been used for this purpose in adults, but the value of this method in pediatric AI has not been established. Forty patients with moderate to severe AI were included in this retrospective study. LV function was assessed by strain analysis and conventional echocardiography both before and after surgery. Of the 32 patients with preserved preoperative ejection fraction (EF; >50 %), 8 had postoperative dysfunction (<50 %). Mean conventional indices of global LV systolic performance for the entire cohort of patients with AI were predominantly in the normal range before surgery. Preoperative values for LV global longitudinal strain (GLS) and strain rate (GLSr) were normal. After surgery, there was a significant decrease in shortening and EF. There was a significant decrease from preoperative to postoperative values for both GLS (-16.07 ± 3.82 vs. -11.06 ± 3.88; p < 0.0001) and GLSr (-0.89 ± 0.24 vs. -0.72 ± 0.27; p = 0.0021). A preoperative GLS of -15.3  (AUC = 0.83, CI = 0.69-0.98, p < 0.0001) and a GLSr of -0.79/s (AUC = 0.86, CI = 0.73-0.98, p < 0.0001) were determined to be predictors of early postoperative dysfunction after surgical repair of moderate to severe AI. A preoperative GLS value of ≤-15.3  and GLSr value of -0.79/s or less are predictors of postoperative ventricular dysfunction, which is defined by EF <50 %. GLS and GLSr value determination may be useful as part of the echocardiographic assessment AI and may help determine the optimal timing of surgery in pediatric patient with at least moderate AI.

    View details for DOI 10.1007/s00246-013-0646-z

    View details for Web of Science ID 000321919400006

    View details for PubMedID 23389100

  • Decremental Left Ventricular Deformation after Pulmonary Artery Band Training and Subsequent Repair in Ventriculoarterial Discordance JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Sun, H. Y., Behzadian, F., Punn, R., Tacy, T. A. 2013; 26 (7): 765-774

    Abstract

    BACKGROUND: Patients with ventriculoarterial discordance, such as congenitally corrected and d-transposition of the great arteries, may undergo a morphologic left ventricular (LV) training strategy consisting of surgical pulmonary artery band (PAB) placement and subsequent anatomic repair to establish ventriculoarterial concordance. The purpose of this study was to characterize morphologic LV function and deformation longitudinally using speckle-tracking strain analysis in patients with ventriculoarterial discordance who underwent LV training. METHODS: Twenty-nine patients (12 with d-transposition of the great arteries and 17 with congenitally corrected transposition of the great arteries) who underwent LV training with PAB placement were evaluated retrospectively. LV ejection fraction and global and regional longitudinal strain and strain rate were measured before and 7 ± 5 days after PAB placement and subsequent anatomic repair. RESULTS: PAB placement caused reductions in the mean LV ejection fraction from 76.1 ± 10.2% to 66.7 ± 7.8% (P < .001), in mean global strain from -17.7 ± 9% to -13.3 ± 7.5% (P = .01), and in mean lateral wall strain from -23.3 ± 12.8% to -17.5 ± 10.3% (P = .01). After anatomic repair (a median of 21 months after PAB placement; range, 0.5-104 months), mean LV ejection fraction decreased further from 63.3 ± 8.6% to 52.4 ± 14.9% (P < .05). Mean global strain declined from -17.6% ± 4.4 to -12.6 ± 4% (P = .01), and mean lateral wall strain decreased from -18.2 ± 11.4% to -12.6 ± 5.3% (P = .04). CONCLUSIONS: In patients with ventriculoarterial discordance undergoing PAB placement for LV training and anatomic repair, the morphologic left ventricle demonstrated decremental systolic function and global longitudinal deformation acutely. Frequent functional assessment is warranted to understand long-term myocardial mechanics in these patients.

    View details for DOI 10.1016/j.echo.2013.03.017

    View details for Web of Science ID 000321051000011

    View details for PubMedID 23623593

  • Aortic valve disease in pulmonary atresia and major aortopulmonary collaterals. Annals of thoracic surgery Mainwaring, R. D., Dimeling, G., Punn, R., Hanley, F. L. 2013; 95 (6): 2122-2125

    Abstract

    Aortic valve disease in association with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals (PA/VSD/MAPCAs) is an extremely rare combination of congenital heart defects. The presence of aortic stenosis or insufficiency or both imposes an additional physiologic burden that complicates the management of PA/VSD/MAPCAs. This report summarizes our experience with 7 patients who underwent surgical repair of this rare combination of defects.This was a retrospective review of patients who had both aortic valve disease and PA/VSD/MAPCAs. Four patients had predominantly aortic stenosis, 2 patients had aortic insufficiency, and 1 had mixed stenosis and insufficiency. Anatomically, this corresponded to a dysplastic trileaflet valve in 4, bicuspid valve in 2, and quadricuspid valve in 1.Four of the 7 patients underwent aortic valve surgery at the time of their first operation for PA/VSD/MAPCAs. The remaining 3 patients had aortic valve surgery at subsequent operations. All 7 patients are alive, with a median duration of follow-up of 3 years. One patient has required aortic valve replacement 2 years after repair of an insufficient bicuspid valve.This manuscript summarizes our experience with 7 patients who had the rare combination of aortic valve disease and PA/VSD/MAPCAs. Although aortic valve disease has been reported for other conotruncal defects, we believe this is the first report of its presence in association with PA/VSD/MAPCAs.

    View details for DOI 10.1016/j.athoracsur.2013.03.070

    View details for PubMedID 23643545

  • Physiologic and molecular characterization of a murine model of right ventricular volume overload. American journal of physiology. Heart and circulatory physiology Reddy, S., Zhao, M., Hu, D., Fajardo, G., Katznelson, E., Punn, R., Spin, J. M., Chan, F. P., Bernstein, D. 2013; 304 (10): H1314-27

    Abstract

    Pulmonary insufficiency (PI) is a common long-term sequel after repair of tetralogy of Fallot, causing progressive right ventricular (RV) dilation and failure. We describe the physiologic and molecular characteristics of the first murine model of RV volume overload. PI was created by entrapping the pulmonary valve leaflets with sutures. Imaging, catheterization, and exercise testing were performed at 1, 3, and 6 mo and compared with sham controls. RNA from the RV free wall was hybridized to Agilent whole genome oligonucleotide microarrays. Volume overload resulted in RV enlargement, decreased RV outflow tract shortening fraction at 1 mo followed by normalization at 3 and 6 mo (39 ± 2, 44 ± 2, and 41 ± 2 vs. 46 ± 3% in sham), early reversal of early and late diastolic filling velocities (E/A ratio) followed by pseudonormalization (0.87 ± 0.08, 0.82 ± 0.08, and 0.96 ± 0.08 vs. 1.04 ± 0.03; P < 0.05), elevated end-diastolic pressure (7.6 ± 0.7, 6.9 ± 0.8, and 7 ± 0.5 vs. 2.7 ± 0.2 mmHg; P < 0.05), and decreased exercise duration (26 ± 0.4, 26 ± 1, and 22 ± 1.3 vs. 30 ± 1.1 min; P < 0.05). Subendocardial RV fibrosis was evident by 1 mo. At 1 mo, 372 genes were significantly downregulated. Mitochondrial pathways and G protein-coupled receptor signaling were the most represented categories. At 3 mo, 434 genes were upregulated and 307 downregulated. While many of the same pathways continued to be downregulated, TNF-?, transforming growth factor-?1 (TGF-?1), p53-signaling, and extracellular matrix (ECM) remodeling transitioned from down- to upregulated. We describe a novel murine model of chronic RV volume overload recapitulating aspects of the clinical disease with gene expression changes suggesting early mitochondrial bioenergetic dysfunction, enhanced TGF-? signaling, ECM remodeling, and apoptosis.

    View details for DOI 10.1152/ajpheart.00776.2012

    View details for PubMedID 23504182

  • Surgical Reconstruction of Pulmonary Stenosis With Ventricular Septal Defect and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Punn, R., Reddy, V. M., Hanley, F. L. 2013; 95 (4): 1417-1421

    Abstract

    Pulmonary stenosis with ventricular septal defect and major aortopulmonary collaterals (PS/VSD/MAPCAs) is an extremely rare form of congenital heart defect. Although it has been assumed that PS/VSD/MAPCAs would be similar to pulmonary atresia (PA) with VSD/MAPCA, there are currently no data to support this conjecture. This study reviewed our surgical experience with reconstruction of PS/VSD/MAPCA.This was a retrospective review of 25 patients (14 boys, 11 girls) who were born with PS/VSD/MAPCA and underwent surgical reconstruction. Preoperative pulmonary angiography was used to define the central branch pulmonary arteries and MAPCA. Patients were a median age of 4 months at the first operation.There was one operative death (4%) in this cohort of 25 patients, and complete repair was achieved in the 24 survivors (96%). There were two distinct subgroups of patients: 11 demonstrated cyanosis in the neonatal timeframe and underwent an initial procedure to augment pulmonary blood flow (+PBF). The remaining 14 patients formed the second group (-PBF). The median age at the first operation was 0.8 months in the +PBF group and 5.2 months in the -PBF group (p<0.005). Complete repair was achieved in 91% of patients in the +PBF group and in 100% in the -PBF group; however, the average number of procedures to achieve complete repair was 2.8 in the +PBF group vs 1.0 in the -PBF group (p<0.005).Outcomes for PS/VSD/MAPCAs as a whole were excellent, with a low surgical mortality and high rate of complete repair. There were two identifiable subgroups with distinctive differences required in their surgical management. These results provide a prognostic outlook for patients with PS/VSD/MAPCAs that can be compared and contrasted with PA/VSD/MAPCAs.

    View details for DOI 10.1016/j.athoracsur.2013.01.007

    View details for Web of Science ID 000317150600039

    View details for PubMedID 23434256

  • Surgical reconstruction of peripheral pulmonary artery stenosis in Williams and Alagille syndromes JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Monge, M. C., Mainwaring, R. D., Sheikh, A. Y., Punn, R., Reddy, V. M., Hanley, F. L. 2013; 145 (2): 476-481

    Abstract

    Peripheral pulmonary artery stenosis is a rare congenital heart defect frequently found in association with Williams and Alagille syndromes. Controversy exists regarding the optimal treatment of peripheral pulmonary artery stenosis, with most centers favoring catheter-based interventions. In contrast, we have preferentially used surgical reconstruction of peripheral pulmonary artery stenosis. The purpose of the present study was to review our experience with surgical reconstruction of peripheral pulmonary artery stenosis.We performed a retrospective review of patients who underwent surgical reconstruction of peripheral pulmonary artery stenosis. A total of 16 patients were identified: 7 had Williams syndrome, 6 had Alagille syndrome, and 3 had no identifiable syndrome. Detailed pulmonary angiography was performed in all patients to define stenoses at the main, branch, lobar, and segmental arterial levels. The mean preoperative right ventricular/left ventricular pressure ratio was 0.88 ± 0.07. The surgical approach was a median sternotomy with cardiopulmonary bypass. All peripheral stenoses were augmented with pulmonary artery homograft tissue. The median age at surgery was 14 months, and concomitant procedures were performed in 9 of the 16 patients.There was 1 operative mortality (6%). The mean right ventricular/left ventricular pressure ratio decreased to 0.40 ± 0.04 postoperatively (P < .005), representing a 55% reduction compared with the preoperative values. The patients were followed up for a median of 5 years. No late mortality occurred and reoperation was not required.The data have demonstrate that this comprehensive surgical approach to the treatment of peripheral pulmonary artery stenosis was associated with low early and no late mortality. Surgical reconstruction of the peripheral pulmonary artery stenosis resulted in a significant decrease in right ventricular pressure. We hypothesize that this reduction in right ventricular pressures will confer a long-term survival advantage for this cohort of patients.

    View details for DOI 10.1016/j.jtcvs.2012.09.102

    View details for Web of Science ID 000313634700030

    View details for PubMedID 23228407

  • Evaluation of Risk Factors for Prediction of Outcome in Fetal Spectrum of Atrioventricular Septal Defects. Congenital heart disease Bader, R. S., Punn, R., Silverman, N. H. 2013

    Abstract

    Atrioventricular septal defects (AVSD) are very commonly diagnosed in utero. Heterotaxy/chromosomal abnormalities frequently coexist with AVSD. However, outcomes of fetal AVSD are not precisely known. We attempted to define mortality risk factors in AVSD.We retrospectively searched our database, electronic records, and echocardiograms with diagnosis of fetal AVSD from 2003 to 2012. We investigated the following risk factors: atrial situs, heart rate/rhythm, ventricular dominance/morphology, atrioventricular valve regurgitation, cardiothoracic ratio, ejection fraction, and extracardiac anomalies.Forty-five fetuses with a median gestational age of 28 weeks (17.5-37.1) were determined to have AVSD during the 10 years, of which 12 were either lost to follow-up (6) or underwent termination (6). There were 16 deaths (48%); two died in utero. Isomerism was identified in 17 of 45 (37%) fetuses (11 left atrial, 6 right atrial isomerism) and chromosomal abnormalities were identified in 12 (27%). Twenty-eight of 33 fetuses, not lost to follow-up or terminated, had extracardiac anomalies which had associated increased mortality (57% vs. 0%, P = .04). Heart block (75% vs. 43%, P = .12), left ventricular noncompaction (80% vs. 43%, P = .17), and isomerism (63% vs. 41%, P = .28) were associated with mortality but without statistical significance. Twenty-five of 45 (56%) had unbalanced AVSD. Positional abnormalities of the great arteries or semilunar valve stenosis were present in 20/45 (44%) while venous anomalies were present in 16/45 (36%). Presence of ventricular dominance, atrioventricular valve regurgitation, elevated cardiothoracic ratio, or diminished ejection fraction were not associated with mortality.Overall mortality rate for fetuses with AVSD was 48%. The presence of extracardiac anomalies is an independent risk factor for prediction of fetal or neonatal demise. Heart block, isomerism, and noncompaction in fetal AVSD appear to be associated with poor outcomes as well but did not reach statistical significance. This information is useful for counseling parents with fetus AVSD.

    View details for DOI 10.1111/chd.12136

    View details for PubMedID 24102719

  • Coronary Sinus Obstruction after Atrioventricular Canal Defect Repair. Congenital heart disease Peng, D. M., Sun, H. Y., Hanley, F. L., Olson, I., Punn, R. 2013

    Abstract

    The coronary sinus can become obstructed with any instrumentation at or near the ostium such as in atrioventricular canal defect repairs. This complication may lead to a wide range of consequences including dyspnea, angina, myocardial infarction, and sudden death. The following report illustrates the importance of careful perioperative echocardiographic evaluation of the coronary sinus in procedures that may affect the sinus and its ostium.

    View details for PubMedID 23682752

  • Predictors of Bronchopulmonary Dysplasia or Death in Premature Infants with a Patent Ductus Arteriosus. Pediatric research Chock, V. Y., Punn, R., Oza, A., Benitz, W. E., Van Meurs, K. P., Whittemore, A. S., Behzadian, F., Silverman, N. H. 2013

    Abstract

    Background:Preterm infants with a PDA are at risk for death or development of BPD. However, PDA treatment remains controversial. We investigated if PDA treatment and other clinical or echocardiographic (ECHO) factors were associated with the development of death or BPD.Methods:We retrospectively studied clinical and ECHO characteristics of preterm infants with birth weight <1500 g and ECHO diagnosis of a PDA. Logistic regression and classification and regression tree (CART) analyses were performed to assess variables associated with the combined outcome of death or BPD.Results:Of 187 preterm infants with a PDA, 75% were treated with indomethacin or surgery and 25% were managed conservatively. Death or BPD occurred in 80 (43%). Logistic regression found lower gestational age (OR 0.5), earlier year of birth during the study period (OR 0.9), and larger ductal diameter (OR 4.3) were associated with the decision to treat the PDA, while gestational age was the only variable associated with death or BPD (OR 0.6, 95% CI 0.5-0.8).Conclusion:Only lower gestational age and not PDA treatment or ECHO score was associated with the adverse outcome of death or BPD. Further investigation of PDA management strategies and effects on adverse outcomes of prematurity is needed.Pediatric Research (2013); doi:10.1038/pr.2013.253.

    View details for DOI 10.1038/pr.2013.253

    View details for PubMedID 24378897

  • Supine Cycling in Pediatric Exercise Testing: Disparity in Performance Measures. Pediatric cardiology May, L. J., Punn, R., Olson, I., Kazmucha, J. A., Liu, M. Y., Chin, C. 2013

    Abstract

    Supine cycling may be an important alternative modality for exercise testing. Subtle differences in supine and upright exercise physiology have been suggested but not fully explored in the pediatric population. The aim of this study was to compare peak and submaximal metabolic data in the upright and supine exercise positions. Healthy children (N = 100) performed cardiopulmonary exercise tests using supine and upright cycle ergometry. Recruitment was governed by grant funding and not based on sample size calculations. Subjects exercised to exhaustion. Paired Student's t-tests were used to compare upright and supine data; simple linear regression analyses examined correlations between the two modalities. Peak heart rate was similar in both testing positions. Although peak oxygen uptake (peak VO2), oxygen uptake at anaerobic threshold (VO2@AT), VO2 when the respiratory exchange ratio is consistently at or above 1.0 (VO2@RER1.0), and the oxygen uptake efficiency slope were significantly higher in the upright position, no difference was considered of practical significance when applying Cohen's effect size criteria. Therefore, it may be reasonable for pediatric exercise laboratories to use established normative data and predictions for upright cycling position and apply them to metabolic measures obtained in supine exercise testing. Supine cycling with submaximal parameters may allow objective assessment of exercise capacity in children who undergo exercise echocardiography or cannot perform upright exercise testing.

    View details for DOI 10.1007/s00246-013-0841-y

    View details for PubMedID 24253612

  • Insights into dyssynchrony in Hypoplastic Left Heart Syndrome HEART RHYTHM Motonaga, K. S., Miyake, C. Y., Punn, R., Rosenthal, D. N., Dubin, A. M. 2012; 9 (12): 2010-2015

    Abstract

    Cardiac resynchronization therapy has been proposed for treatment of hypoplastic left heart syndrome (HLHS) patients with right ventricular (RV) failure. The role of dyssynchrony, however, is poorly understood in this population.The purpose of this study was to better understand the relationship between electrical and mechanical dyssynchrony in HLHS using 3-dimensional electrical mapping, tissue Doppler indices of wall motion, and vector velocity imaging.Eleven HLHS subjects with normal RV function and ten normal subjects (age 3-18 years) were studied. Electrical and mechanical activation times and dyssynchrony indices (electrical dyssynchrony index, mechanical dyssynchrony index) were calculated using 3-dimensional electrical mapping, tissue Doppler indices, and vector velocity imaging.No differences in measures of electrical dyssynchrony were seen when comparing HLHS patients and normal patients (electrical activation time 63.3 ± 22.8 ms vs 56.2 ± 11.2 ms, P = .38; electrical dyssynchrony index 13.7 ± 6.3 ms vs 11.6 ± 3.0 ms, P = .34). However, measures of mechanical dyssynchrony were markedly abnormal in HLHS patients despite normal RV function (mechanical activation time 16 ± 11.3 ms vs 0.9±1.9 ms, P = .01; mechanical dyssynchrony index 7.5 ± 5.5 vs 0.4 ± 0.8, P<.01).Patients with HLHS and preserved RV systolic function have normal electrical activation when compared to patients with normal right and left ventricles. In contrast, these patients demonstrate mechanical dyssynchrony compared to patients with normal right and left ventricles. This finding raises important questions about the indications for cardiac resynchronization therapy in this patient population.

    View details for DOI 10.1016/j.hrthm.2012.08.031

    View details for Web of Science ID 000311791900029

    View details for PubMedID 23085485

  • Supine Exercise Echocardiographic Measures of Systolic and Diastolic Function in Children JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Punn, R., Obayashi, D. Y., Olson, I., Kazmucha, J. A., Depucci, A., Hurley, M. P., Chin, C. 2012; 25 (7): 773-781

    Abstract

    Echocardiography has been used to determine ventricular function, segmental wall motion abnormality, and pulmonary artery pressure before and after peak exercise. No prior study has investigated systolic and diastolic function using echocardiography at various phases of exercise in children. The aim of this study was to determine the fractional shortening (FS), systolic-to-diastolic (S/D) ratio, heart rate-corrected velocity of circumferential fiber shortening (VCFc), circumferential wall stress (WS), ratio of mitral passive inflow to active inflow (E/A), ratio of passive inflow by pulsed-wave to tissue Doppler (E/E'), and right ventricular-to-right atrial pressure gradient from tricuspid valve regurgitation jet velocity (RVP) and time duration at various phases of exercise in children.In an 8-month period (December 2007 to July 2008), 100 healthy children were evaluated, and 97 participants aged 8 to 17 years who performed complete cardiopulmonary exercise stress tests using supine cycle ergometry were prospectively enrolled. The participants consisted of 48 female and 49 male subjects with various body sizes, levels of exercise experience, and physical capacities. The cardiopulmonary exercise stress test consisted of baseline pulmonary function testing, continuous gas analysis and monitoring of blood pressure and heart rate responses, electrocardiographic recordings, and oxygen saturation measurement among participants who pedaled against a ramp protocol based on body weight. All participants exercised to exhaustion. Echocardiography was performed during exercise at baseline, at a heart rate of 130 beats/min, at a heart rate of 160 beats/min, at 5 min after exercise, and at 10 min after exercise. FS, S/D ratio, VCFc, WS, E/A, E', E/E', and RVP at these five phases were compared in all subjects.All echocardiographic parameters differed at baseline from 160 beats/min (P < .0001) except E/E', which remained at 5.4 to 5.8. Specifically, FS (from 37% to 46%), S/D ratio, VCFc (from 1.1 to 1.6), WS (from 200 to 258 g/cm(2)), E' (from 0.2 to 0.3), and RVP (from 18 to 35 mm Hg) increased from baseline to 160 beats/min and then subsequently decreased to at or near baseline, while tricuspid valve regurgitation duration decreased (from 370 to 178 msec).Normal values for systolic and diastolic echocardiographic measurements of function are now available. FS, VCFc, WS, and RVP increase with exercise and then return to near baseline levels. The E/E' ratio is unaltered with exercise in normal subjects.

    View details for DOI 10.1016/j.echo.2012.03.007

    View details for Web of Science ID 000305701600013

    View details for PubMedID 22521368

  • Tissue Doppler-Derived Measurement of Isovolumic Myocardial Contraction in the Pediatric Population PEDIATRIC CARDIOLOGY Punn, R., Behzadian, F., Tacy, T. A. 2012; 33 (5): 720-727

    Abstract

    Multiple echocardiographic techniques have been utilized to quantify systolic function. The shortening and ejection fraction remain the most commonly used and accepted methods. However, these measures are affected by altered loading conditions, and are not applicable when ventricular geometry differs from the prolate ellipsoid typical of a left ventricle. Mitral valve annular acceleration during isovolumic contraction (IVA) has been proposed as a load independent index of left ventricular contractility. However, published values for IVA demonstrating normal function vary. In addition, the value of IVA which may discern impaired systolic function has not been established. The purpose of this study is to determine a threshold IVA value for abnormal left ventricular function in the pediatric population. Structurally/functionally normal control (n = 90) and dilated cardiomyopathy (study = 64) patients were compared for differences in left ventricular: wall stress (WS), velocity of circumferential fiber shortening (VCFc), ejection fraction (EF), ejection force, and pulsed wave-derived medial and lateral wall IVA. No difference in body surface area (p = 0.61) or gender (p = 0.53) was noted. Left ventricular ejection fraction, ejection force, VCFc, and IVA were significantly lower and WS was significantly higher in the study group (p < 0.01). The medial IVA was 1.71 ± 0.89 m/s(2) for an EF <40%, 1.74 ± 0.70 m/s(2) for an EF = 40-50%, 2.46 ± 0.89 m/s(2) for an EF >50%. The lateral IVA was 1.81 ± 1.03 m/s(2) for an EF <40%, 2.07 ± 0.78 m/s(2) for an EF = 40-50%, 2.54 ± 0.99 m/s(2) for an EF >50%. ROC analysis demonstrated a medial IVA of 1.97 m/s(2) as the cut-off for predicting an EF <50% with a 77% sensitivity of and specificity of 66% (AUC = 0.75, CI = 0.67-0.83, p < 0.01). ROC analysis demonstrated a lateral IVA of 2.31 m/s(2) as the cut-off for predicting an EF <50% with a 73% sensitivity of and specificity of 63% (AUC = 0.72, CI = 0.63-0.82, p < 0.01). IVA lateral of 1.93 m/s(2) or less was associated with heart transplant and death. ICC analysis demonstrated some interobserver variability in IVA measurement (0.57-0.65). The normal IVA of the medial and lateral mitral valve annulus measure over 1.97 m/s(2) and 2.31 m/s(2), respectively; values less than this cut-off are associated with EF <50%. Despite some problems with reproducibility IVA remains a promising method of screening for diminished ventricular contractility in the setting of abnormal geometry.

    View details for DOI 10.1007/s00246-012-0200-4

    View details for Web of Science ID 000304458900006

    View details for PubMedID 22349669

  • Imaging of the Aorta: Embryology and Anatomy SEMINARS IN ULTRASOUND CT AND MRI Murillo, H., Lane, M. J., Punn, R., Fleischmann, D., Restrepo, C. S. 2012; 33 (3): 169-190

    Abstract

    This review focuses on the embryology and anatomy of the aorta with some imaging examples. Dividing the aorta by segments of unique function and embryogenesis facilitates organizing the group of potential anomalies encountered. A basic understanding of the embryologic development of the aorta and its major branches helps in understanding and recognizing typical and atypical anatomic findings. Diagnostic imaging of the aorta and its major branches can be accomplished by invasive and noninvasive methods, based on the clinical scenario and the age of the patient. In this review, computed tomography and magnetic resonance imaging examples are emphasized.

    View details for DOI 10.1053/j.sult.2012.01.013

    View details for Web of Science ID 000305094700002

    View details for PubMedID 22624964

  • Late Repair of the Native Pulmonary Valve in Patients With Pulmonary Insufficiency After Surgery for Tetralogy of Fallot ANNALS OF THORACIC SURGERY Mainwaring, R. D., Pirolli, T., Punn, R., Hanley, F. L. 2012; 93 (2): 677-679

    Abstract

    Pulmonary regurgitation developing late after tetralogy of Fallot repair is now recognized as a serious threat to the long-term welfare of these patients. This article summarizes our experience with 5 patients who underwent reoperations for treatment of severe pulmonary regurgitation after transannular patch repair of tetralogy of Fallot. In each case, the intraoperative findings revealed anatomy favorable for valve repair and enabled preservation of the native pulmonary valves.

    View details for DOI 10.1016/j.athoracsur.2011.09.016

    View details for Web of Science ID 000299540200069

    View details for PubMedID 22269747

  • Echocardiographic Diagnosis and Prognosis of Fetal Left Ventricular Noncompaction JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Arunamata, A., Punn, R., Cuneo, B., Bharati, S., Silverman, N. H. 2012; 25 (1): 112-120

    Abstract

    Left ventricular noncompaction (LVNC) has rarely been described in the fetus.The presence of associated congenital heart disease and rhythm disturbance was identified and the presence of heart failure was assessed using the cardiovascular profile score in all fetuses with LVNC presenting from January 1999 to July 2010. The left ventricle was divided into 12 segments-four segments each at the base, midpapillary, and apical regions-in the short-axis view to calculate the noncompaction/compaction ratio for each segment.Of 24 fetuses with LVNC included in the study, 22 had significant congenital heart disease, and 15 had complete heart block. Of the 16 patients with adequate follow-up and not electively terminated, 12 (81%) died or progressed to heart transplantation. The average noncompaction/compaction ratios were 2.02 in patients who died or underwent heart transplantation and 1.67 in survivors (P = .2034). Fifty-seven of 93 measured segments (61%) of the left ventricle in the patients who died or underwent heart transplantation had noncompaction/compaction ratios ? 2 compared with five of 17 measured segments (29%) in survivors (P = .0837). The average cardiovascular profile score was 6. The apical region had greater involvement of noncompaction than the midpapillary and basal regions, with ratios of 2.27, 2.14, and 1.10, respectively (P = .00035).Fetuses with LVNC have a poor prognosis that may be related to associated congenital heart disease, increased segmental involvement of noncompaction, and complete heart block and can be predicted by the cardiovascular profile score.

    View details for DOI 10.1016/j.echo.2011.09.019

    View details for Web of Science ID 000298276500013

    View details for PubMedID 22014428

  • Novel Electrocardiogram Manifestations of a Tension Pneumothorax PEDIATRIC CARDIOLOGY Punn, R., Seslar, S. P. 2011; 32 (8): 1258-1259

    View details for DOI 10.1007/s00246-011-0067-9

    View details for Web of Science ID 000296733100039

    View details for PubMedID 21811813

  • Surgical Results in Patients With Pulmonary Atresia-Major Aortopulmonary Collaterals in Association With Total Anomalous Pulmonary Venous Connection ANNALS OF THORACIC SURGERY Mainwaring, R. D., Reddy, M., Reinhartz, O., Punn, R., Tacy, T., Hanley, F. L. 2011; 92 (5): 1756-1760

    Abstract

    Pulmonary atresia and major aortopulmonary collaterals (PA/MAPCAs) is a complex form of congenital heart disease. One to two percent of patients with PA/MAPCAs will also have total anomalous pulmonary venous connection (TAPVC). This study summarizes our surgical experience with this rare combination of life-threatening congenital heart defects.A retrospective review was performed to identify patients who had surgery for PA/MAPCAs in association with TAPVC. From November 2001 to March 2011, 9 patients presented with this combination of defects. Eight of the 9 patients had heterotaxy with an unbalanced atrioventricular canal and functional single ventricle. The ninth patient had double outlet right ventricle (two ventricles). Timing of surgical intervention was typically predicated on the degree of pulmonary venous obstruction. The median age at surgery was 14 days. All nine patients had surgical correction of TAPVC, unifocalization of MAPCA's into a central confluence, and placement of a shunt.There was one early mortality (< 30 days) and two late mortalities. For the 6 survivors, 5 have subsequently undergone a bidirectional Glenn procedure, and 3 had completion of their Fontan. Two patients are currently at the bidirectional Glenn stage; one is a good candidate for Fontan completion while the other is not suitable. The sixth patient is awaiting further assessment.The PA/MAPCAs, in association with TAPVC, is a challenging combination of defects. The data suggest that the combination of PA/MAPCAs and TAPVC can be undertaken with a reasonable midterm prognosis.

    View details for DOI 10.1016/j.athoracsur.2011.06.020

    View details for Web of Science ID 000296925400045

    View details for PubMedID 21944736

  • QTc prolongation in children following congenital cardiac disease surgery CARDIOLOGY IN THE YOUNG Punn, R., Lamberti, J. J., Balise, R. R., Seslar, S. P. 2011; 21 (4): 400-410

    Abstract

    IntroductionQTc prolongation has been reported in adults following cardiopulmonary bypass; however, this phenomenon has not been studied in children with congenital cardiac disease. This study's aim was to formally assess QTc in children undergoing cardiac surgery.Pre-operative and post-operative electrocardiograms during hospital stays were prospectively analysed on 107 consecutive patients under 18 years of age undergoing cardiac surgery. QTc was measured manually in leads II, V4, and V5. Measurements of 440 and 480 milliseconds were used to categorise patients. Peri-procedural data included bypass and cross-clamp time, medications, and electrolyte measurements. Outcome data included arrhythmias, length of mechanical ventilation, and hospital stay. Patients with post-operative new bundle branch block or ventricularly paced rhythm were excluded.In all, 59 children were included, out of which 26 had new QTc over 440 milliseconds and 6 of 59 had new QTc over 480 milliseconds post-operatively. The mean increase in post-operative QTc was 25 milliseconds, p=0.0001. QTc over 480 was associated with longer cross-clamp time, p=0.003. Other risk factors were not associated with post-operative QTc prolongation. This phenomenon was transient with normalisation occurring in 67% of patients over 60 hours on average. One patient with post-operative QTc over 440 milliseconds developed ventricular tachycardia. There was no correlation between prolonged QTc and duration of mechanical ventilation, or hospital stay.ConclusionA significant number of children undergoing cardiac surgery showed transient QTc prolongation. The precise aetiology of QT prolongation was not discerned, though new QTc over 480 milliseconds was associated with longer cross-clamp time. In this cohort, transient QTc prolongation was not associated with adverse sequela.

    View details for DOI 10.1017/S1047951111000175

    View details for Web of Science ID 000293593800005

    View details for PubMedID 21362209

  • Fetal Predictors of Urgent Balloon Atrial Septostomy in Neonates with Complete Transposition JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Punn, R., Silverman, N. H. 2011; 24 (4): 425-430

    Abstract

    In complete transposition of the great vessels, a restrictive patent foramen ovale leads to inadequate circulatory mixing and severe cyanosis. Urgent balloon atrial septostomy (BAS) improves mixing and bridges neonates to surgery. Several studies have determined risk factors in utero for poor postnatal outcomes in complete transposition of the great vessels, particularly a restrictive patent foramen ovale and ductus arteriosus. In addition to these risk factors, we studied two new features, a hypermobile septum and reverse diastolic patent ductus arteriosus shunt, to determine which patients will require an urgent BAS.We reviewed all 26 fetuses from 2001 to 2010 with complete transposition of the great vessels and closely examined the patent foramen ovale and septum primum for hypermobility, restriction, flat appearance, or redundancy. We defined hypermobility as a septum primum flap that oscillates between both atria. We also examined the ductus size and shunting pattern to evaluate whether these features contributed to urgent BAS.In total, 14 of 26 fetuses required urgent BAS with improved cyanosis. Nine fetuses had an urgent BAS and a hypermobile septum, and 12 fetuses had no urgent BAS or hypermobile septum. Eight fetuses had an urgent BAS and a reverse diastolic patent ductus arteriosus, and 11 fetuses had no urgent BAS or reverse diastolic patent ductus arteriosus. A hypermobile septum and reverse diastolic patent ductus arteriosus had a significant association with urgent BAS (P < .01, sensitivity = 0.64 and 0.57, specificity = 1.0 and 0.92, positive predictive value = 1.0 and 0.89, negative predictive value = 0.71 and 0.65). No fetus had a restrictive patent foramen ovale/ductus arteriosus.A hypermobile septum and reverse diastolic patent ductus arteriosus are new prenatal findings to help predict the need for an urgent BAS postnatally in patients with complete transposition of the great vessels.

    View details for DOI 10.1016/j.echo.2010.12.020

    View details for Web of Science ID 000288781700011

    View details for PubMedID 21324642

  • The Echocardiographic Evaluation of Left Ventricular Non-compaction Pediatric Ultrasound Today Punn R, Silverman N 2011; 16 (6): 109-28
  • Annular Tilt as a Screening Test for Right Ventricular Enlargement in Patients with Tetralogy of Fallot JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Punn, R., Behzadian, F., Tacy, T. A. 2010; 23 (12): 1297-1302

    Abstract

    Right ventricular end-diastolic volume (RVEDV) greater than 150 mL/m² is a risk factor for sudden death in patients with tetralogy of Fallot (TOF) after repair. Because of its anterior placement and abnormal geometry, two-dimensional echocardiography is limited to a qualitative assessment of RVEDV. Cardiac magnetic resonance imaging (CMRI) and computed tomography angiography (CTA) are the accepted standards for quantifying RVEDV. This study evaluated the ability of a novel echocardiographic measure, the right ventricular annular tilt (RVAT), to identify patients with increased RVEDV.All patients with repaired TOF with an echocardiogram and CMRI or CTA were included in this retrospective study. The RVAT was determined by measuring the angle of the tricuspid valve plane relative to the mitral valve plane at end-diastole in the apical 4-chamber view in study (n = 38) and age-matched control (n = 74) patients. The RVEDV measurements were obtained by CMRI (n = 32) or CTA (n = 6). The study and control patients' ages were no different (11.3 and 11.8 years, P = .73).The study group RVAT was significantly higher than the control group RVAT (17.4 vs. 0.1 degrees; P < .0001). RVAT values greater than 20 degrees had a mean RVEDV of 166 ± 60 mL/m², whereas RVAT less than 20 degrees had a mean RVEDV of 122 ± 25 mL/m² (P = .0370). Receiver operating characteristic analysis demonstrated an RVAT of 17.9 degrees as the cutoff for predicting a RVEDV of greater than 150 mL/m² with a sensitivity of 75% and specificity of 73% (area under the curve = 0.76; confidence interval, 0.56-0.96; P = .0063). Intraclass correlation analysis demonstrated minimal interobserver and intraobserver variability when measuring RVAT (0.99 and 0.92).An RVAT less than 20 degrees is associated with an RVEDV less than 150 mL/m². RVAT is a useful echocardiographic technique for detecting increased RVEDV in patients with TOF and may help discern which patients should undergo RVEDV quantification by CMRI or CTA.

    View details for DOI 10.1016/j.echo.2010.09.002

    View details for Web of Science ID 000284624900012

    View details for PubMedID 20950999

  • Newborn with Persistent Truncus Arteriosus and Interrupted Aortic Arch Demonstrating Reverse Left Subclavian Artery Flow PEDIATRIC CARDIOLOGY Chan, E. L., Tacy, T. A., Punn, R. 2010; 31 (8): 1254-1256

    View details for DOI 10.1007/s00246-010-9817-3

    View details for Web of Science ID 000284157400025

    View details for PubMedID 20957476

  • Anomalies Associated With a Prominent Azygos Vein on Echocardiography in the Pediatric Population JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Punn, R., Olson, I. 2010; 23 (3): 282-285

    Abstract

    Prominent azygos veins (PAVs) have been described with interrupted inferior venae cavae (IVCs) with heterotaxy. At the authors' institution, cases of PAVs with uninterrupted IVCs have been noted. The aim of this study was to determine the occurrence rate of PAVs and associated lesions by echocardiography.All patients with PAVs were collected; those with interrupted and uninterrupted IVCs were assigned to groups 1 and 2. Normal controls were assigned to group 3.Among 15,849 patients from January 1, 2001, to March 31, 2008, 55 (0.3%) had PAVs, 42 (76%) in group 1 and 13 (24%) in group 2. Heterotaxy was prominent in group 1, whereas patients in group 2 had no heterotaxy. Patients in group 2 had more structurally normal hearts than those in group 1 (46% vs 14%, P<.01), partial anomalous pulmonary venous return, and one single ventricle. IVC measurements were the same in groups 2 and 3 (P=.65).This study demonstrates that a PAV without IVC interruption is not associated with heterotaxy. Patients with PAVs should be carefully examined for partial anomalous pulmonary venous return.

    View details for DOI 10.1016/j.echo.2009.11.025

    View details for Web of Science ID 000275221900007

    View details for PubMedID 20138465

  • Cardiac Segmental Analysis in Left Ventricular Noncompaction: Experience in a Pediatric Population JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Punn, R., Silverman, N. H. 2010; 23 (1): 46-53

    Abstract

    Echocardiography has been used to diagnose and describe left ventricular noncompaction (LVNC). No other study has investigated LVNC using the 16-segment model described by the American Heart Association and the American Society of Echocardiography in children, some of whom have congenital heart disease. Using the ratio of noncompaction to compaction, the authors analyzed the 16 segments and determined if severity was correlated with poor outcomes in a pediatric population.The 16-segment noncompaction/compaction ratio, shortening, and ejection fractions were measured retrospectively in all children with LVNC at a single institution from January 1, 2000, to June 30, 2008.Forty-four patients had LVNC, an incidence of 0.3% of laboratory admissions. Twenty-eight patients (64%) who remained alive were assigned to group 1, and 16 patients (36%) who either died or were transplanted constituted group 2. Group 2 had more patients with significant associated congenital heart disease than group 1 (50% vs 18%, P < .05). We found similar regions of involvement in the 16-segment model with sparing of basal segments and involvement of the midpapillary and apical regions (P < .001); however, patients in group 2 were noted to have more segments involved (6 vs 4, P < .05), lower shortening fractions (16% vs 29%, P < .001), and lower ejection fractions (24% vs 47%, P < .001). The ejection fraction was inversely related to the number of segments (r = -0.63, P < .01), suggesting that more noncompaction portends a worse outcome.In younger patients with noncompaction, poor outcomes such as low ejection fractions, death, and transplantation are related to the number of left ventricular segments involved. There is more associated congenital heart disease in the pediatric population, which carries a poorer prognosis than the disease reported in adult populations.

    View details for DOI 10.1016/j.echo.2009.09.003

    View details for Web of Science ID 000273052600011

    View details for PubMedID 19857942

  • Echocardiography in patients with left ventricular inflow lesions. Pediatric Ultrasound Today. Punn R, Silverman NH 2008; 13 (1): 3-23
  • Case report: Rhabdomyolysis induced by mibefradil in a patient treated with cyclosporine and simvastatin JOURNAL OF CLINICAL PHARMACOLOGY Wombolt, D. G., Jackson, A., Punn, R., Smith, S., McCune, T. R., Williams, P. B. 1999; 39 (3): 310-312

    View details for Web of Science ID 000078782800015

    View details for PubMedID 10073331

Conference Proceedings


  • Surgical outcomes for patients with pulmonary atresia/major aortopulmonary collaterals and Alagille syndrome Mainwaring, R. D., Sheikh, A. Y., Punn, R., Reddy, V. M., Hanley, F. L. OXFORD UNIV PRESS INC. 2012: 235-241

    Abstract

    Pulmonary atresia with major aortopulmonary collateral arteries (PA/MAPCAs) is a complex congenital heart defect that has undergone significant advances in treatment over the past 15 years. A small subset of patients with PA/MAPCAs have associated Alagille syndrome, which can have an adverse impact on many other organ systems. The purpose of this study was to review our institutional outcomes for the surgical patients with PA/MAPCAs and Alagille syndrome.This was a retrospective review of patients with PA/MAPCA's and Alagille who underwent surgical reconstruction from November 2001 to August 2011. Fifteen patients were identified in our data base. Thirteen had pulmonary atresia with ventricular septal defect (PA/VSD) and two had pulmonary atresia with intact ventricular septum (PA-IVS).There has been no early or late mortality in this cohort of 15 patients with PA/MAPCA' and Alagille syndrome. The patients have undergone a total of 38 cardiac surgical procedures. Ten of the 13 patients with PA/VSD have achieved complete repair, including unifocalization, a right ventricle to pulmonary artery conduit and closure of all intra-cardiac shunts. The three unrepaired patients with PA/VSD remain potential candidates for eventual complete repair, while the two patients with PA-IVS remain viable candidates for a single ventricle pathway. The patients in this series have also undergone 12 major non-cardiac procedures.The data demonstrate that surgical reconstruction of PA/MAPCAs can be successfully achieved in patients with Alagille syndrome. The longer-term prognosis remains guarded on the basis of the multi-organ system involvement of Alagille syndrome.

    View details for DOI 10.1093/ejcts/ezr310

    View details for Web of Science ID 000306365600013

    View details for PubMedID 22402453

  • Is There a Rote for Cardiac Resynchronization Therapy in Pediatric Pulmonary Hypertension? Motonaga, K. S., MIYAKE, C. Y., Punn, R., Rosenthal, D. N., Feinstein, J. A., Dubin, A. M. ELSEVIER SCIENCE INC. 2012: S29-S29

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