Bio

Clinical Focus


  • Pediatric Cardiology

Academic Appointments


Professional Education


  • Medical Education:Pritzker School of Medicine University of Chicago Registrar (2005) IL
  • Fellowship:Baylor College of Medicine Registrar (2011) TX
  • Board Certification: Pediatrics, American Board of Pediatrics (2009)
  • Board Certification: Pediatric Cardiology, American Board of Pediatrics (2012)
  • Fellowship:Texas Children's Hospital Baylor College of Medicine (2012) TX
  • Residency:Stanford University Medical Center (2008) CAUnited States of America

Publications

All Publications


  • Weakly supervised classification of rare aortic valve malformations using unlabeled cardiac MRI sequences Nature Communications Fries, J. A., Varma, P., Chen, V. S., Xiao, K., Tejeda, H., Saha, P., Dunnmon, J., Chubb, H., Maskatia, S., Fiterau, M., Delp, S., Ashley, E., Ré, C., Priest, J. R. 2019; 10
  • Fetal left-sided cardiac structural dimensions in left-sided congenital diaphragmatic hernia - association with severity and impact on postnatal outcomes PRENATAL DIAGNOSIS Kailin, J. A., Dhillon, G. S., Maskatia, S. A., Cass, D. L., Shamshirsaz, A. A., Mehollin-Ray, A. R., Cassady, C. I., Ayres, N. A., Wang, Y., Belfort, M. A., Olutoye, O. O., Ruano, R. 2017; 37 (5): 502-509

    Abstract

    Fetuses with congenital diaphragmatic hernia (CDH) demonstrate varying degrees of left heart hypoplasia. Our study assesses the relationship between fetal left-sided cardiac structural dimensions, lung size, percentage liver herniation, lung-to-head ratio, postnatal left-sided cardiac structural dimensions, and postnatal outcomes.We performed a retrospective cohort study of fetuses with left-sided CDH who had prenatal echocardiographic, ultrasound, and magnetic resonance imaging examinations at our institution between January 2007 and March 2015. Postnatal outcomes assessed include use of inhaled nitric oxide (iNO), use of extracorporeal membrane oxygenation, and death.Fifty-two fetuses with isolated left-sided CDH were included. Multivariate logistic regression models indicated that smaller fetal aortic valve z-score was associated with postnatal use of iNO (p = 0.03). Fetal mitral valve z-score correlated with lung-to-head ratio (p = 0.04), postnatal mitral valve z-score correlated with percent liver herniation (p = 0.03), and postnatal left ventricular end-diastolic dimension z-score correlated with liver herniation <20% (p = 0.04).We identified associations between smaller fetal left-sided cardiac structural dimensions and classic CDH indices. Smaller aortic valve z-score was associated with iNO use; however, left heart dimensions showed no association with extracorporeal membrane oxygenation or mortality. Further study into the impact of left-sided hypoplasia on outcomes in CDH is worthy of evaluation in a larger, prospective study. © 2017 John Wiley & Sons, Ltd.

    View details for DOI 10.1002/pd.5045

    View details for Web of Science ID 000401561200012

    View details for PubMedID 28370263

  • Outcomes After Decompression of the Right Ventricle in Infants With Pulmonary Atresia With Intact Ventricular Septum Are Associated With Degree of Tricuspid Regurgitation: Results From the Congenital Catheterization Research Collaborative CIRCULATION-CARDIOVASCULAR INTERVENTIONS Petit, C. J., Glatz, A. C., Qureshi, A. M., Sachdeva, R., Maskatia, S. A., Justino, H., Goldberg, D. J., Mozumdar, N., Whiteside, W., Rogers, L. S., Nicholson, G. T., McCracken, C., Kelleman, M., Goldstein, B. H. 2017; 10 (5)

    Abstract

    Outcomes after right ventricle (RV) decompression in infants with pulmonary atresia with intact ventricular septum vary widely. Descriptions of outcomes are limited to small single-center studies.Neonates undergoing RV decompression for pulmonary atresia with intact ventricular septum were included from 4 pediatric centers. Primary end point was reintervention post-RV decompression; secondary end points included circulation type at latest follow-up. Ninety-nine patients (71 with pulmonary atresia with intact ventricular septum and 28 with virtual atresia) underwent RV decompression at median 3 (25th-75th, 2-5) days of age. Seventy-one patients (72%) underwent at least 1 reintervention after decompression. Median duration of follow-up was 3 years (range, 1-10). Freedom from reintervention was 51% at 1 month and 23% at 3 years. In multivariable analysis, reintervention was associated with virtual atresia (hazard ratio [HR], 0.51; 95% confidence interval [CI], 0.28-091; P=0.027), smaller RV length (HR, 0.94; 95% CI, 0.89-0.99; P=0.027), and ≤mild tricuspid regurgitation (TR; HR, 3.58; 95% CI, 2.04-6.30; P<0.001). Patients undergoing surgical shunt or ductal stent were less likely to have virtual atresia (HR, 0.36; 95% CI, 0.15-0.85; P=0.02) and more likely to have higher RV end-diastolic pressure (HR, 1.07; 95% CI, 1.00-1.15; P=0.057) and ≤mild TR (HR, 3.50; 95% CI, 1.75-7.0; P<0.001). Number of reinterventions was associated with ≤mild TR (rate ratio, 1.87; 95% CI, 1.23-2.87; P=0.0037). Multivariable analysis indicated that <2-ventricle circulation status was associated with ≤mild TR (odds ratio, 18.6; 95% CI, 5.3-65.2; P<0.001) and lower RV area (odds ratio, 0.81; 95% CI, 0.72-0.91; P<0.001).Patients with pulmonary atresia with intact ventricular septum deemed suitable for RV decompression have a high reintervention burden although most achieve 2-ventricle circulation. TR ≤mild at baseline is strongly associated with reintervention and <2-ventricle circulation at medium-term follow-up. Degree of baseline TR may be an important marker of long-term outcomes in this population.

    View details for DOI 10.1161/CIRCINTERVENTIONS.116.004428

    View details for Web of Science ID 000401546000004

    View details for PubMedID 28500137

  • Left ventricular rotational mechanics in early infancy: Normal reference ranges and reproducibility of peak values and time to peak values. Early human development Maskatia, S. A., Lee, W., Altman, C. A., Ayres, N. A., Feagin, D. K., Pignatelli, R. H. 2017; 104: 39-44

    Abstract

    Left ventricular cardiac twist and torsion values have been described in premature and term neonates, but not in early infancy. Early and late peak untwist rates and time to peak (TTP) values have not been described in infants.53 term infants were enrolled prospectively. The following parameters were obtained by two blinded observers at 1-2months postnatal age: peak twist and torsion (twist indexed to LV length), peak twist rate and torsion rate, TTP twist, early peak untwist rate, TTP early untwist rate, late peak untwist rate, TTP late untwist rate. Reproducibility was assessed using intraclass correlation and Bland Altman analysis.Intraclass correlation was ≥0.87 for all peak rotational mechanics values. Measures of TTP values had intraclass correlation (ICC) values ≤0.77, with TTP twist rate demonstrating the lowest ICC (0.69). The only measure which demonstrated significant bias was TTP twist rate. Peak twist demonstrated modest correlation (R=0.52, p<0.001) with global circumferential strain, and no correlation with ejection fraction, global longitudinal strain, or left ventricular myocardial performance index.Measurements of rotational mechanics and timing to peak values have acceptable reproducibility. Peak twist, twist rate, and early untwist rate values in early infancy are similar to those reported in premature neonates, and higher than those reported in older children. Twist indexed to LV length (torsion) is lower in early infancy than in premature neonates, but higher than in term neonates.

    View details for DOI 10.1016/j.earlhumdev.2016.11.004

    View details for PubMedID 28042971

  • Echocardiographic Parameters and Outcomes in Primary Fetal Cardiomyopathy JOURNAL OF ULTRASOUND IN MEDICINE Ezon, D. S., Ayres, N. A., Altman, C. A., Denfield, S. W., Morris, S. A., Maskatia, S. A. 2016; 35 (9): 1949-1955

    Abstract

    Primary fetal cardiomyopathy is a rare entity, with a poor prognosis. We sought to describe its echocardiographic characteristics and outcomes.We performed a 12-year retrospective review of fetuses with primary cardiomyopathy.Of more than 6000 fetuses evaluated, 25 met criteria for primary cardiomyopathy, and 18 had sufficient echocardiographic and pregnancy outcome data for inclusion. At echocardiography, the median gestational age was 29.6 weeks (range, 21.0-36.4 weeks); median cardiovascular profile score was 6 (range, 1-9); median right ventricular Tei index was 0.52 (range, 0.32-0.94); and median left ventricular Tei index was 0.40 (range, 0.15-0.88). Two had fetal demise, and 16 survived to delivery. The median cardiovascular profile score in those with fetal demise was 3.0 and in those who survived to delivery was 6.5 (range, 3-9; P = .14). The median right ventricular Tei index in those with fetal demise was 0.39 and in those surviving to delivery was 0.53 (range, 0.38-0.94; P = .49). The median left ventricular Tei index in those with fetal demise was 0.29 and in those surviving to delivery was 0.42 (range, 0.15-0.88; P = .50). Sixty-day survival was available in 11 of 16 fetuses. In addition to the 2 with fetal demise, 4 had postnatal demise, and 5 were alive at a median follow-up of 39 months. Hydrops (P = .01), skin edema (P = .01), and mild or greater mitral regurgitation (P = .02) were associated with fetal or postnatal demise, with a trend toward an association between moderate or greater tricuspid regurgitation (P = .07) and fetal or postnatal demise.Hydrops and atrioventricular valvar regurgitation are ominous signs in primary fetal cardiomyopathy. Although other commonly used methods for assessing cardiovascular performance may help in diagnosing primary cardiomyopathy, these data suggest limited predictive value.

    View details for DOI 10.7863/ultra.15.05059

    View details for PubMedID 27466259

  • Pilot study of chronic maternal hyperoxygenation and effect on aortic and mitral valve annular dimensions in fetuses with left heart hypoplasia ULTRASOUND IN OBSTETRICS & GYNECOLOGY Lara, D. A., Morris, S. A., Maskatia, S. A., Challman, M., Nguyen, M., FEAGIN, D. K., Schoppe, L., Zhang, J., Bhatt, A., Sexson-Tejtel, S. K., Lopez, K. N., Lawrence, E. J., Andreas, S., Wang, Y., Belfort, M. A., Ruano, R., Ayres, N. A., Altman, C. A., Aagaard, K. M., Becker, J. 2016; 48 (3): 365-372

    Abstract

    Acute maternal hyperoxygenation (AMH) results in increased fetal left heart blood flow. Our aim was to perform a pilot study to determine the safety, feasibility and direction and magnitude of effect of chronic maternal hyperoxygenation (CMH) on mitral and aortic valve annular dimensions in fetuses with left heart hypoplasia (LHH) after CMH.Gravidae with fetal LHH were eligible for inclusion in a prospective evaluation of CMH. LHH was defined as: sum of aortic and mitral valve annuli Z-scores < -4.5, arch flow reversal and left-to-right or bidirectional atrial level shunting without hypoplastic left heart syndrome or severe aortic stenosis. Gravidae with an affected fetus and with ≥ 10% increase in aortic/combined cardiac output flow after 10 min of AMH at 8 L/min 100% fraction of inspired oxygen were offered enrollment. Nine gravidae were enrolled from February 2014 to January 2015. The goal therapy was ≥ 8 h daily CMH from enrollment until delivery. Gravidae who were cared for from July 2012 to October 2014 with fetal LHH and no CMH were identified as historical controls (n = 9). Rates of growth in aortic and mitral annuli over the final trimester were compared between groups using longitudinal regression.There were no significant maternal or fetal complications in the CMH cohort. Mean gestational age at study initiation was 29.6 ± 3.2 weeks for the intervention group and 28.4 ± 1.8 weeks for controls (P = 0.35). Mean relative increase in aortic/combined cardiac output after AMH was 35.3% (range, 18.1-47.9%). Median number of hours per day on CMH therapy was 9.3 (range, 6.5-14.6) and median duration of CMH was 48 (range, 33-84) days. Mean mitral annular growth was 0.19 ± 0.05 mm/week compared with 0.14 ± 0.05 mm/week in CMH vs controls (mean difference 0.05 ± 0.05 mm/week, P = 0.33). Mean aortic annular growth was 0.14 ± 0.03 mm/week compared with 0.13 ± 0.03 mm/week in CMH vs controls (mean difference 0.01 ± 0.03 mm/week, P = 0.75). More than 9 h CMH daily (n = 6) was associated with better growth of the aortic annulus in intervention fetuses (0.16 ± 0.03 vs 0.08 ± 0.02 mm/week, P = 0.014).CMH is both safe and feasible for continued research. In this pilot study, the effect estimates of annular growth, using the studied method of delivery and dose of oxygen, were small. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

    View details for DOI 10.1002/uog.15846

    View details for PubMedID 26700848

  • Fetal and Neonatal Diastolic Myocardial Strain Rate: Normal Reference Ranges and Reproducibility in a Prospective, Longitudinal Cohort of Pregnancies JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Maskatia, S. A., Pignatelli, R. H., Ayres, N. A., Altman, C. A., Sangi-Haghpeykar, H., Lee, W. 2016; 29 (7): 663-669

    Abstract

    Normative fetal diastolic strain rate values have not been comprehensively reported. The aims of this study were to report normative data for diastolic strain rate parameters across gestation and upon delivery, determine the effect of advancing gestational age on these measures, and compare interobserver variability at multiple gestational age time points.Sixty gravid women were enrolled before 20 weeks' gestation. The following measures were obtained by two blinded observers at five time points across gestation and at 4 to 8 weeks' postnatal age: global left ventricular circumferential strain rate peak E and A waves, global left and right ventricular longitudinal strain rate peak E and A waves, and mitral and tricuspid valve E/A ratios. Reproducibility was assessed using intraclass correlation and Bland-Altman analysis. Least square means analysis was used to evaluate for changes across gestational age.Left ventricular longitudinal and circumferential diastolic strain rate values decreased across gestation, while right ventricular longitudinal values remained stable. With delivery, left ventricular values remained fairly stable, while right ventricular values increased. Intraclass correlations for diastolic strain rate values were 0.68 to 0.94 at ≥24 weeks' gestation and 0.25 to 0.82 for values at 20 to 21 weeks' gestation. Intraclass correlations ranged from 0.49 to 0.90 for mitral valve and tricuspid valve E/A ratios across gestation.Fetal measures of diastolic strain rate have acceptable reproducibility after 24 weeks' gestation. The described changes across gestation likely reflect intrinsic myocardial adaptation to loading conditions. These reference ranges can be used to assess effects of various disease states on fetal myocardial deformation.

    View details for DOI 10.1016/j.echo.2016.02.017

    View details for PubMedID 27049665

  • Association of Late Gadolinium Enhancement and Degree of Left Ventricular Hypertrophy Assessed on Cardiac Magnetic Resonance Imaging With Ventricular Tachycardia in Children With Hypertrophic Cardiomyopathy AMERICAN JOURNAL OF CARDIOLOGY Spinner, J. A., Noel, C. V., Denfield, S. W., Krishnamurthy, R., Jeewa, A., Dreyer, W. J., Maskatia, S. A. 2016; 117 (8): 1342-1348

    Abstract

    There are limited data on the clinical significance of left ventricular (LV) mass and late gadolinium enhancement (LGE) in pediatric hypertrophic cardiomyopathy (HC). We reviewed cardiovascular magnetic resonance (CMR) studies of children with HC to investigate the associations between the extent and distribution of LGE and LV mass with ventricular tachycardia (VT) in children with HC. A blinded observer reviewed CMR studies for the presence and distribution of LV hypertrophy and LGE using a 17-segment model. The primary outcome was VT. LGE was present 17 of 33 subjects (52%). VT was present on outpatient Holter monitor or exercise stress test in 7 patients, of which 5 patients (71%) had LGE. Each additional segment of LGE was associated with an increase in the odds of VT (odds ratio [OR] 1.4, 95% CI 1.1 to 1.9) and fewer than 5 segments with LGE had 93% specificity for the presence or absence of VT (OR 0.06, 95% CI 0.01 to 0.5). VT was more common in patients with LGE in the apical septal (p = 0.03), basal inferoseptal (p <0.01), and basal inferior (p = 0.04) segments, whereas LGE in more commonly involved segments (midanteroseptal and midinferoseptal) was not associated with VT (p = 0.13, 0.26). Patients with VT had greater LV mass index (76.4 ± 40.4 g/m(2.7) vs 50.9 ± 24.3 g/m(2.7); p = 0.03). Each centimeter of increased maximum LV thickness was associated with increased likelihood of VT (OR 2.9, 95% CI 1.2 to 6.8). In conclusion, in pediatric HC, CMR to evaluate the extent and pattern of LGE, LV mass index, and maximum LV thickness may help to identify children with HC at risk of VT.

    View details for DOI 10.1016/j.amjcard.2016.01.032

    View details for PubMedID 26892450

  • Longitudinal Changes and Interobserver Variability of Systolic Myocardial Deformation Values in a Prospective Cohort of Healthy Fetuses across Gestation and after Delivery JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Maskatia, S. A., Pignatelli, R. H., Ayres, N. A., Altman, C. A., Sangi-Haghpeykar, H., Lee, W. 2016; 29 (4): 341-349

    Abstract

    Normative data for fetal myocardial deformation values have not been comprehensively described in a longitudinal cohort. The effect of gestational age on these values and on interobserver variability require further investigation.Sixty gravid women were prospectively enrolled before 20 weeks' gestation. The following measures were obtained by two blinded observers at five time points across gestation and also at 4 to 8 weeks' postnatal age: global circumferential strain and strain rate, global longitudinal left ventricular strain and strain rate, global longitudinal right ventricular strain and strain rate, and left and right ventricular myocardial performance indices. Optimal myocardial visualization and frame rate (≥100 frames/sec) were ensured.For gestational age groups ≥24 weeks, intraclass correlation coefficients between observers were >0.70 for all measures and >0.85 for the majority of measures of myocardial deformation. At 20 to 21 weeks' gestation, intraclass correlation coefficients were 0.35 to 0.52 for longitudinal measures and 0.74 to 0.82 for circumferential measures. Myocardial performance index intraclass correlation coefficients were <0.80 at all time points and <0.70 for most time points. Global longitudinal left ventricular strain and global circumferential strain values remained stable across gestational age groups. Global longitudinal right ventricular strain values remained stable across gestation and increased after birth. Global circumferential strain rate, global longitudinal left ventricular strain rate, and global longitudinal right ventricular strain rate decreased from 20 to 21 weeks' gestation to the remainder of gestation and then remained stable until delivery. Upon delivery, global circumferential strain rate and global longitudinal left ventricular strain rate decreased, and global longitudinal right ventricular strain rate increased.Interobserver variability of fetal strain and strain rate measured at ≥24 weeks' gestation was lower in comparison with values obtained at 20 to 21 weeks' gestation and lower in comparison with left ventricular and right ventricular myocardial performance indices using the described protocol. Gestational changes in fetal myocardial deformation values likely reflect changes in preload and/or afterload on the fetal heart.

    View details for DOI 10.1016/j.echo.2015.12.003

    View details for PubMedID 26797677

  • Complex Surgical Repair of a Flail Tricuspid Valve After Chest Wall Trauma in a Pediatric Patient ANNALS OF THORACIC SURGERY Loar, R. W., Maskatia, S. A., McLaughlin, E. S., Mott, A. R., Adachi, I., Fraser, C. D. 2016; 101 (3): E65-E67

    Abstract

    Traumatic injury to the tricuspid valve can result from severe nonpenetrating chest wall trauma. We present the case of an initially asymptomatic 11-year-old girl who was kicked in the chest by a horse. The trauma resulted in avulsed papillary muscles, ruptured chordae, and right heart failure. She underwent early tricuspid valve reconstruction and annuloplasty. We advocate for routine use of echocardiography to assess for traumatic injury to intracardiac structures. In addition, we believe that early surgical intervention may prevent right-sided heart dysfunction, atrial arrhythmias, and the need for valve replacement.

    View details for DOI 10.1016/j.athoracsur.2015.09.041

    View details for PubMedID 26897232

  • Estimated Combined Cardiac Output Aids in the Assessment of Fetuses with Twin-Twin Transfusion Syndrome Echocardiography, In press Maskatia, S. 2016
  • Cardiovascular magnetic resonance techniques and findings in children with myocarditis: a multicenter retrospective study JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE Banka, P., Robinson, J. D., Uppu, S. C., Harris, M. A., Hasbani, K., Lai, W. W., Richmond, M. E., Fratz, S., Jain, S., Johnson, T. R., Maskatia, S. A., Lu, J. C., Samyn, M. M., Patton, D., Powell, A. J. 2015; 17

    Abstract

    Cardiovascular magnetic resonance (CMR) is increasingly used to diagnose myocarditis in adults but its use in children is not well-established. We sought to describe the presentation, CMR protocol and findings, and outcomes in a multicenter cohort of children with myocarditis.Thirteen hospitals retrospectively identified patients meeting the following inclusion criteria: 1) diagnosis of myocarditis by the managing physicians, 2) age <21 years, 3) CMR examination within 30 days of presentation, and 4) no congenital heart disease. Clinical data and test results, including CMR findings, were abstracted from the medical record.For the 143 patients meeting inclusion criteria, the median age was 16.0 years (range, 0.1-20.3) and 139 (97 %) were hospitalized at the time of CMR. The median time from presentation to CMR was 2 days (0-28). The median left ventricular ejection fraction at CMR was 56 % (10-74), with 29 (20 %) below 45 %. The median right ventricular ejection fraction was 54 % (15-72), with 11 (8 %) below 40 %. There was significant variability among centers in the types of tissue characterization techniques employed (p < 0.001). Overall, late gadolinium enhancement (LGE) was used in 100 % of studies, followed by T2-weighted imaging (T2W) in 69 %, first-pass contrast perfusion (FPP) in 48 %, and early gadolinium enhancement (EGE) in 28 %. Abnormalities were most common with LGE (81 %), followed by T2W (74 %), EGE (55 %), and FPP (8 %). The CMR study was interpreted as positive for myocarditis in 117 patients (82 %), negative in 18 (13 %), and equivocal in 7 (5 %), yielding a sensitivity of 82 %. At a median follow-up of 7.1 months (0-87), all patients were alive and 5 had undergone cardiac transplantation. CMR parameters at presentation associated with persistent left ventricular dysfunction were larger left ventricular end-diastolic volume and lower left and right ventricular ejection fraction but not abnormal LGE.Despite significant practice variation in imaging protocol among centers, CMR had a high sensitivity for the diagnosis of myocarditis in pediatric patients. Abnormalities were most often seen with LGE followed by T2W, EGE, and FPP. These findings should be useful in designing future prospective studies.

    View details for DOI 10.1186/s12968-015-0201-6

    View details for PubMedID 26576638

  • Older Age at Completion of Fontan Procedure Is Associated with Improved Percentage of Predicted Maximum Oxygen Uptake TEXAS HEART INSTITUTE JOURNAL Bolin, E. H., Maskatia, S. A., Tate, A. L., Petit, C. J. 2015; 42 (4): 333-340

    Abstract

    We tested the hypothesis that later completion of the Fontan procedure is associated with improved exercise capacity in the current period of staged single-ventricle palliation. We performed a retrospective study, in Fontan patients, of exercise stress test data from April 2003 through March 2011. Patients were included if they had received staged palliations in accordance with current surgical strategy, defined as the performance of a superior cavopulmonary connection at ≤1 year of age, followed in subsequent years by Fontan completion. Patients with a pacemaker or respiratory exchange ratio <1 were excluded. Early and late Fontan groups were created on the basis of whether Fontan completion had been performed at <4 or ≥ 4 years of age. The primary predictor variable was age at Fontan completion, and the primary marker of exercise performance was the percentage of predicted maximum oxygen consumption. During the study period, 55 patients were identified (mean age, 11.7 ± 2.8 yr). Older age at Fontan completion correlated positively with higher percentages of predicted maximum oxygen consumption (R=0.286, P=0.034). Patients in whom Fontan completion was performed at ≥4 years of age had higher percentages of predicted maximum oxygen consumption than did those in whom completion was at <4 years of age (84.4 ± 21.5 vs 72.9 ± 18.1; P=0.041). Later Fontan completion might be associated with improved exercise capacity in patients palliated in accordance with contemporary surgical strategy.

    View details for DOI 10.14503/THIJ-14-4178

    View details for Web of Science ID 000360211900007

    View details for PubMedID 26413015

  • Congenital heart anomaly in newborns with congenital diaphragmatic hernia: a single-center experience ULTRASOUND IN OBSTETRICS & GYNECOLOGY Ruano, R., Javadian, P., Kailin, J. A., Maskatia, S. A., Shamshirsaz, A. A., Cass, D. L., Zamora, I. J., Sangi-Haghpeykar, H., Lee, T. C., Ayres, N. A., Mehollin-Ray, A., Cassady, C. I., Fernandes, C., Welty, S., Belfort, M. A., Olutoye, O. O. 2015; 45 (6): 683-688

    Abstract

    To evaluate the impact of the presence of a congenital heart anomaly (CHA) and its potential contribution to morbidity and mortality in infants with congenital diaphragmatic hernia (CDH).In this retrospective cohort study, prenatal and postnatal data of all newborns diagnosed with CDH between January 2004 and December 2012 in a single center were reviewed. Cases were classified into two groups: those with 'isolated' CDH and those with both CDH and CHA. Patients with CHA were further subclassified into those with a major or minor CHA based on the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STS-EACTS) scoring systems. Patients with associated non-cardiac anomalies, including 'syndromic cases', were excluded from the analysis. Primary and secondary outcomes were survival up to 1 year of age and a need for extracorporeal membrane oxygenation (ECMO), respectively.Of the 180 infants with CDH, 41 were excluded because of the presence of non-cardiac associated anomalies, 118 had isolated CDH and 21 had CDH with CHA (16 with minor and five with major CHA). Receiver-operating characteristics curve analysis demonstrated that the best cut-off for survival was when the score for CHA was ≤ 2 for both RACHS-1 (area under the curve (AUC), 0.74 (P = 0.04); sensitivity, 80.0%; specificity, 87.5%) and STS-EACTS (AUC, 0.83 (P = 0.03); sensitivity, 100%; specificity, 87.5%). Survival rate at 1 year was significantly lower in those with CHD and a major CHA (40.0%; P = 0.04) than in those with isolated CDH (77.1%) and those with CDH and a minor CHA (81.3%). We found no significant differences among the groups with regard to the need for ECMO.In general, a milder form of CHA does not appear to have a negative impact on the survival of infants with CDH. However, mortality appears to be significantly higher in infants with CDH and a major form of CHA. The scoring systems appear to be useful as predictors for classifying the effects of CHA in this population of patients.

    View details for DOI 10.1002/uog.14648

    View details for Web of Science ID 000355674700011

    View details for PubMedID 25158239

  • Echocardiographic Parameters of Right Ventricular Diastolic Function in Repaired Tetralogy of Fallot Are Associated with Important Findings on Magnetic Resonance Imaging CONGENITAL HEART DISEASE Maskatia, S. A., Morris, S. A., Spinner, J. A., Krishnamurthy, R., Altman, C. A. 2015; 10 (3): E113-E122

    Abstract

    Right ventricular (RV) dilation, RV dysfunction, RV outflow tract obstruction, and branch pulmonary artery stenosis are risk factors associated with the need for future surgical or catheter-based intervention commonly assessed by cardiac magnetic resonance in patients with tetralogy of Fallot who have undergone initial repair. The ability of echocardiography to predict the presence of these risk factors is limited.We aimed to determine the association between echocardiographic parameters of right ventricular diastolic dysfunction and risk factors for intervention as identified on cardiac magnetic resonance imaging (CMR).We reviewed all echocardiograms and CMRs performed at our institution within 1 year of each other in patients with repaired tetralogy of Fallot. We evaluated patients for the following: RV systolic dysfunction (RV ejection fraction <45%), RV dilation (right ventricular end-diastolic volume ≥ 160 mL/m(2)), and RV obstruction (RV outflow tract gradient ≥ 3 m/s by echocardiogram or branch pulmonary artery stenosis assessed by CMR). We evaluated for associations between the above CMR findings and echocardiographic parameters of diastolic function.CMR and echocardiographic images performed between March 2007 and March 2012 were available in 99 patients. RV obstruction was associated with lower E/A ratio (P = .02), E/A reversal (P = .02), and prograde end diastolic pulmonary flow (P < .01). Low tricuspid annular a' and low septal s' were associated with lower RV ejection fraction (P < .01, P < .01). Elevated E/e' was associated with higher RV end diastolic volume (P = .04). In predicting the presence of any of the evaluated risk factors for future intervention, TV E/e' ≥ 4.7 had positive predictive value (PPV) of 86% and negative predictive value (NPV) of 50%, TV E/A reversal had PPV of 89% and NPV of 34, and prograde end diastolic pulmonary flow on echocardiogram had PPV of 91% and NPV of 38%.Echocardiographic parameters of diastolic dysfunction have a reasonable PPV but poor NPV for predicting clinically significant risk factors identified by CMR.

    View details for DOI 10.1111/chd.12265

    View details for PubMedID 25916551

  • Clinical validation of free breathing respiratory triggered retrospectively cardiac gated cine balanced steady-state free precession cardiovascular magnetic resonance in sedated children JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE Krishnamurthy, R., Pednekar, A., Atweh, L. A., Vogelius, E., Chu, Z. D., Zhang, W., Maskatia, S., Masand, P., Morris, S. A., Krishnamurthy, R., Muthupillai, R. 2015; 17

    Abstract

    Cine balanced steady-state free precession (SSFP), the preferred sequence for ventricular function, demands uninterrupted radio frequency (RF) excitation to maintain the steady-state during suspended respiration. This is difficult to accomplish in sedated children. In this work, we validate a respiratory triggered (RT) SSFP sequence that drives the magnetization to steady-state before commencing retrospectively cardiac gated cine acquisition in a sedated pediatric population.This prospective study was performed on 20 sedated children with congenital heart disease (8.6 ± 4 yrs). Identical imaging parameters were used for multiple number of signal averages (MN) and RT cine SSFP sequences covering both the ventricles in short-axis (SA) orientation. Image quality assessment and quantitative volumetric analysis was performed on the datasets by two blinded observers. One-sided Wilcoxon signed rank test and Box plot analysis were performed to compare the clinical scores. Bland-Altman (BA) analysis was performed on LV and RV volumes.Scan duration for SA stack using RT-SSFP (3.9 ± 0.8 min) was slightly shorter than MN-SSFP (4.6 ± 0.9 min) acquisitions. The endocardial edge definition was significantly better for RT than MN, blood to myocardial contrast was better for RT than MN without reaching statistical significance, and inter slice alignment was comparable. BA analysis indicates that the variability of volumetric indices between RT and MN is comparable to inter and intra-observer variability reported in the literature.The free breathing RT-SSFP sequence allows diagnostic images in sedated children with significantly better edge definition when compared to MN-SSFP, without any penalty for total scan time.

    View details for DOI 10.1186/s12968-014-0101-1

    View details for Web of Science ID 000349945400001

    View details for PubMedID 25589308

  • Marked Septal Dyskinesis From Wolff-Parkinson-White Syndrome CIRCULATION Niu, M. C., Maskatia, S. A., Kim, J. J. 2014; 130 (23): E196-E198
  • Surgical management of tetralogy of fallot: in defense of the infundibulum. Seminars in thoracic and cardiovascular surgery McKenzie, E. D., Maskatia, S. A., Mery, C. 2013; 25 (3): 206-212

    Abstract

    Surgical treatment of the Tetralogy of Fallot (ToF) is one of the great successes of medicine and also a topic of controversy. Different strategies have been proposed, including age-based (neonatal) management strategies as well as anatomic-based management strategies. Regardless of the management strategy entailed, the surgical management of ToF has considerably evolved over the years. As a result, patients can now expect excellent early results with survival approaching 100% for those without genetic syndromes. The goals of current surgical therapy should be to mitigate the late right ventricular (RV) dysfunction that may occur by minimizing the extent of surgical injury during the intial repair. As the surgical techniques continue to advance, the outcomes will continue to improve.

    View details for DOI 10.1053/j.semtcvs.2013.10.003

    View details for PubMedID 24331142

  • Repeat Balloon Aortic Valvuloplasty Effectively Delays Surgical Intervention in Children With Recurrent Aortic Stenosis CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Petit, C. J., Maskatia, S. A., Justino, H., Mattamal, R. J., Crystal, M. A., Ing, F. F. 2013; 82 (4): 549-555

    Abstract

    Balloon aortic valvuloplasty (BAV) is the primary therapy for congenital aortic stenosis (AS). Recurrent AS following initial BAV or initial surgical valvotomy (SV) may require a second BAV (BAV2). We sought to determine the longterm outcomes of BAV2.We reviewed all cases of BAV2, defined as BAV following primary BAV or SV between 1988 and 2009. Cases were reviewed for pre- and post-BAV2 echocardiographic and procedural details.Tertiary care dedicated children's hospital.Between 1985 and 2009, 43 patients underwent BAV2 (23 primary SV, 20 primary BAV) at median age 1.9 years (1 month-21 years) and median weight 15 (3.3-55) kg.BAV2 performed following primary SV or primary BAV.We evaluated the following endpoints: ≥ moderate AI post-BAV2, aortic valve replacement (AVR), additional BAV or SV post-BAV2, death and heart transplantation.The gradient decreased from 61.4 ± 16.0 mm Hg to 26.0 ± 13.6 post-BAV2 (P < 0.01). Gradient prior to BAV2 was higher in primary SV patients (66 ± 13 mm Hg) than in primary BAV patients (56 ± 18 mm Hg, P = 0.04). 24 patients had no further events after BAV2, while 19 patients (44%) experienced 23 events including: AVR (n = 8), SV (n = 6), BAV3 (n = 2), death (n = 5), and transplant (n = 1). Regression demonstrated that adverse events were associated with higher post-BAV2 gradient (P < 0.01). Repeat intervention on the aortic valve and AVR were associated with higher post BAV2 gradient (P = 0.04, P = 0.01). Prior to BAV2, 7 patients (17%) had AI > mild, compared to 21 (51%) patients after BAV2. Cox regression revealed that primary BAV was associated with development of AI > mild after BAV2 (P < 0.01).BAV2 is associated with decreased valve gradient, though with an increase in AI. However, residual AS, not AI, is associated with poor outcomes following BAV2. BAV2 effectively treats recurrent AS and postpones need for surgical intervention.

    View details for DOI 10.1002/ccd.24562

    View details for Web of Science ID 000329285200036

    View details for PubMedID 22815228

  • The Echocardiography "Boot Camp": A Novel Approach in Pediatric Cardiovascular Imaging Education JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Maskatia, S. A., Altman, C. A., Morris, S. A., Cabrera, A. G. 2013; 26 (10): 1187-1192

    Abstract

    Dynamic training schedules introduce novel challenges to medical specialty training programs that require manual dexterity. The aim of this study was to examine the effect of a 3-day, intensive pediatric echocardiography course ("boot camp") on trainee self-efficacy and on the acquisition and short-term retention of basic echocardiographic knowledge and skills for first-year pediatric cardiology fellows (CFs).The boot camp consisted of hands-on structured practice guided by sonographers and cardiology faculty members, didactic lectures, and reading. Pre-boot camp experience was assessed using an experience score. Outcome measures included written precamp and postcamp examinations, a performance-based test, precamp and postcamp self-efficacy assessments, and the number and quality of echocardiographic examinations performed in the first 3 months of fellowship.Six CFs completed the boot camp. Two of the six CFs reported experience scores of 2 out of 10, whereas the remainder reported experience scores of 0 out of 10. Performance-based test scores ranged from 68 to 99 out of 147. All six CFs reported precamp self-efficacy scores of 21 (the minimum score), compared with median postcamp scores of 82 (range, 49-94) (P = .01). Scores on the written examination improved from median of 16 (range, 11-18) to 23.5 (range, 22-28) (P = .01). CFs who completed the boot camp completed 28 independent echocardiographic examinations (median, 4 per CF) during the first 3 months of fellowship, an increase from six independent examinations (median, 1 per CF) by CFs during the year before institution of the boot camp (P = .030). Echocardiograms obtained by CFs who had completed the boot camp scored higher on total quality (P = .004), overall two-dimensional image quality (P = .011), functional assessments (P = .015), and assessment for pericardial effusion (P = .031).The echocardiography boot camp improves self-efficacy in performing an echocardiographic examination and the acquisition and short-term retention of skills and knowledge required to perform pediatric echocardiography.

    View details for DOI 10.1016/j.echo.2013.06.001

    View details for Web of Science ID 000324748300011

    View details for PubMedID 23860091

  • Impact of Obesity on Ventricular Size and Function in Children, Adolescents and Adults With Tetralogy of Fallot After Initial Repair AMERICAN JOURNAL OF CARDIOLOGY Maskatia, S. A., Spinner, J. A., Nutting, A. C., Slesnick, T. C., Krishnamurthy, R., Morris, S. A. 2013; 112 (4): 594-598

    Abstract

    Obesity is epidemic in congenital heart disease, with reported rates of 16% to 26% in children and 54% in adults. The aim of this study was to evaluate the impact of obesity on ventricular function and size in patients after initial repair for tetralogy of Fallot (TOF). Cardiac magnetic resonance studies in normal-weight (body mass index percentile <85th) and obese (body mass index percentile ≥95th) children and adults with repaired tetralogy of Fallot were reviewed. The left ventricular ejection fraction, the right ventricular ejection fraction, left and right ventricular end-diastolic volumes indexed to actual body surface area, to height, and to body surface area using ideal body weight were evaluated in 36 obese patients and 72 age-matched normal-weight patients. Compared with normal-weight patients, obese patients had lower right ventricular ejection fractions (mean 46 ± 9% vs 51 ± 7%, p = 0.003) and left ventricular ejection fractions (mean 57 ± 9% vs 61 ± 6%, p = 0.017), higher right ventricular end-diastolic volumes indexed to height (mean 160 ± 59 vs 135 ± 41 ml/m, p = 0.015) and left ventricular end-diastolic volumes indexed to height (mean 86 ± 25 vs 70 ± 20 ml/m, p = 0.001), and higher right ventricular end-diastolic volumes indexed to ideal body weight (mean 166 ± 55 vs 144 ± 38 ml/m², p = 0.020) and left ventricular end-diastolic volumes indexed to ideal body weight (mean 90 ± 22 vs 75 ± 15 ml/m², p <0.001). In conclusion, obesity is a modifiable risk factor associated with worsened biventricular systolic function and biventricular dilation in patients with repaired tetralogy of Fallot. The standard method of indexing ventricular volumes using actual body surface area may underestimate volume load in obese patients.

    View details for DOI 10.1016/j.amjcard.2013.04.030

    View details for Web of Science ID 000323407700023

    View details for PubMedID 23677064

  • Effect of Branch Pulmonary Artery Stenosis on Right Ventricular Volume Overload in Patients With Tetralogy of Fallot After Initial Surgical Repair AMERICAN JOURNAL OF CARDIOLOGY Maskatia, S. A., Spinner, J. A., Morris, S. A., Petit, C. J., Krishnamurthy, R., Nutting, A. C. 2013; 111 (9): 1355-1360

    Abstract

    Right ventricular (RV) volume overload secondary to pulmonary regurgitation is common in patients after initial repair of tetralogy of Fallot (TOF) and is associated with adverse long-term outcomes. The objective of the present study was to determine the effect of branch pulmonary artery stenosis on the RV volume in patients with repaired TOF. We reviewed 178 cardiac magnetic resonance imaging studies in patients with repaired TOF. We defined bilateral stenosis as a Nakata index of ≤200 mm(2)/m(2) and concordant branch pulmonary artery cross-sectional area, unilateral stenosis as 1 branch pulmonary artery cross-sectional area ≤100 mm(2)/m(2) and 1 branch pulmonary artery cross-sectional area >100 mm(2)/m(2), and restrictive physiology as prograde main pulmonary artery diastolic flow. Of the 178 patients, 20 (11%) had bilateral stenosis, 47 (26%) unilateral stenosis, and 111 (63%) had no stenosis. The RV end-diastolic volume was lower in patients with bilateral (125 ± 27 ml/m(2)) or unilateral (131 ± 43 ml/m(2)) stenosis than in those without stenosis (149 ± 35 ml/m(2), p = 0.021 and p = 0.019, respectively). The main pulmonary artery regurgitant fraction was greater in patients without stenosis (47%, range 2% to 69%) than in those with bilateral (33%, range 9% to 59%; p = 0.009) or unilateral stenosis (40%, range 0% to 71%; p = 0.033). Restrictive physiology was more common in patients with bilateral (13 of 15, 87%) or unilateral (21 of 38, 55%) stenosis than in those without stenosis (28 of 85, 33%; p <0.001 and p = 0.017, respectively). In conclusion, in patients with repaired TOF, bilateral and unilateral branch pulmonary artery stenosis was associated with a greater main pulmonary artery regurgitant fraction and smaller RV end-diastolic volume than those in patients without stenosis, likely owing to the development of restrictive physiology. Branch pulmonary artery stenosis might effectively delay the referral for pulmonary valve replacement.

    View details for DOI 10.1016/j.amjcard.2013.01.278

    View details for Web of Science ID 000318827900020

    View details for PubMedID 23411101

  • Aortic valve morphology is associated with outcomes following balloon valvuloplasty for congenital aortic stenosis CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Maskatia, S. A., Justino, H., Ing, F. F., Crystal, M. A., Mattamal, R. J., Petit, C. J. 2013; 81 (1): 90-95

    Abstract

    Evaluate the incidence of various morphologic types of congenital AS, and the association between valve morphology and long-term outcomes, including repeat BAV, AVR, and death/transplant.Reports on long-term outcomes have low agreement on the influence of morphologic type.We queried our institutional database and hospital billing records to identify all patients who underwent balloon aortic valvuloplasty (BAV) from 1992 through 2009. We excluded cases where morphology was not clear based on the description in the pre-BAV echocardiogram report and patients who underwent single ventricle palliation. The primary outcome of the study was the occurrence of any of the following events: repeat valvuloplasty, AVR, heart transplant, or death.There were 147 patients in our study cohort. The most common morphology was functionally bicuspid (n = 92, 63%), followed by functionally unicuspid (n = 20, 14%), dysplastic (n = 16, 11%), true bicuspid (n = 13, 9%), and true unicuspid (n = 6, 4%). The primary endpoint was less likely to occur in patients with functionally bicuspid valves (P < 0.01) and patients with true bicuspid valves (P = 0.03), whereas it was more likely to occur in patients with functionally unicuspid valves (P = 0.02) and patients with true unicuspid valves (P = 0.05). Multivariate Cox regression analysis demonstrated that valve type other than functionally bicuspid was associated with diminished freedom from repeat intervention, death or transplant (HR 3.3, CI 1.2 - 8.6, P = 0.02).In our cohort, patients with functionally bicuspid aortic valves, the most common type, had improved outcomes as compared with all other morphologic types.

    View details for DOI 10.1002/ccd.24286

    View details for Web of Science ID 000312942300033

    View details for PubMedID 23044747

  • Morphologic Heterogeneity in Left Ventricular Noncompaction Resulting in Accessory Left Ventricular Chambers CIRCULATION-HEART FAILURE Ezon, D., Maskatia, S., Jeewa, A., Denfield, S. 2012; 5 (6): E94-E95
  • Pulmonary reperfusion injury after the unifocalization procedure for tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Maskatia, S. A., Feinstein, J. A., Newman, B., Hanley, F. L., Roth, S. J. 2012; 144 (1): 184-189

    Abstract

    The aims of our study are to describe the incidence, clinical profile, and risk factors for pulmonary reperfusion injury after the unifocalization procedure for tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries. We hypothesized the following: (1) Pulmonary reperfusion injury is more likely to occur after unifocalization procedures in which a septated circulation is not achieved, (2) pulmonary reperfusion injury is directly related to the severity of stenosis in major aortopulmonary collateral arteries, and (3) pulmonary reperfusion injury leads to longer intubation time and longer hospitalization.Consecutive patients with tetralogy of Fallot/pulmonary atresia/major aortopulmonary collateral arteries who underwent unifocalization procedures over a 5-year period were identified in our institutional database. Chest radiographs before the unifocalization procedure, from postoperative days 0 to 4, and from 2 weeks after the unifocalization procedure or at discharge were evaluated by a pediatric radiologist for localized pulmonary edema. Determination of stenosis severity was based on review of preoperative angiograms. Statistical analyses using multivariate repeated-measures analyses were performed with generalized estimating equations.Pulmonary reperfusion injury was present after 42 of 65 (65%) unifocalization procedures. In 36 of 42 cases of reperfusion injury, unilateral injury was present. Risk factors for the development of reperfusion injury included bilateral unifocalization (P = .01) and degree of stenosis (P = .03). We did not identify an association between pulmonary reperfusion injury and time to tracheal extubation or hospital discharge.Pulmonary reperfusion injury is common after the unifocalization procedure for tetralogy of Fallot/pulmonary atresia/major aortopulmonary collateral arteries. Severity of stenosis and bilateral unifocalization are associated with the development of reperfusion injury.

    View details for DOI 10.1016/j.jtcvs.2011.12.030

    View details for Web of Science ID 000305412200031

    View details for PubMedID 22244564

  • Type B Interrupted Left Aortic Arch with Isolated Right Subclavian Artery CONGENITAL HEART DISEASE Knudson, J. D., Lopez, K. N., Maskatia, S., McKenzie, E. D., Lantin-Hermoso, M. R., Masand, P. M., Vick, G. W. 2012; 7 (3): E25-E30

    Abstract

    Interrupted aortic arch is a rare congenital heart malformation occurring in approximately three per 1 million births. Type B interrupted aortic arch (interruption between the second carotid artery and the ipsilateral subclavian artery) is the most common of three major types (A, B, and C). We report an extremely rare finding: a case of left-sided type B interrupted aortic arch with isolation of the right subclavian artery (origin from the right pulmonary artery).

    View details for DOI 10.1111/j.1747-0803.2011.00625.x

    View details for Web of Science ID 000304437100007

    View details for PubMedID 22309184

  • Hypertrophic Cardiomyopathy: Infants, Children, and Adolescents CONGENITAL HEART DISEASE Maskatia, S. A. 2012; 7 (1): 84-92

    Abstract

    Hypertrophic cardiomyopathy (HCM) is characterized by inappropriate left ventricular hypertrophy (LVH) in the setting of a nondilated left ventricle. HCM is often associated with asymmetric LVH, a family history of HCM, sarcomeric genetic mutations, and an increased risk of sudden cardiac death. There is a wide clinical variability in HCM presenting during childhood and a relative lack of data on the pediatric population. This review will cover HCM presenting in infancy, childhood, and adolescence.

    View details for DOI 10.1111/j.1747-0803.2011.00613.x

    View details for Web of Science ID 000299466700016

    View details for PubMedID 22222117

  • Restrictive Physiology is Associated With Poor Outcomes in Children With Hypertrophic Cardiomyopathy PEDIATRIC CARDIOLOGY Maskatia, S. A., Decker, J. A., Spinner, J. A., Kim, J. J., Price, J. F., Jefferies, J. L., Dreyer, W. J., Smith, E. O., Rossano, J. W., Denfield, S. W. 2012; 33 (1): 141-149

    Abstract

    Pediatric patients with hypertrophic cardiomyopathy (HCM) and restrictive physiology (RP) with poor outcomes have been identified, but data on their course are limited. Our goal was to delineate the clinical features and course of children with HCM and RP. An institutional review of 119 patients identified between 1985 and 2010 with the diagnosis of HCM was performed. The diagnosis of RP was based on >1 echocardiogram along with at least one of the following: left atrial enlargement without evidence of left ventricle dilation, E/E' ratio ≥ 10, and E/A ratio ≥ 3. Outcomes analysis was performed using Cox or Poisson regression when appropriate. RP was present in 50 (42%) patients. In patients without RP, 10-year freedom-from-death or aborted sudden cardiac death (aSCD), and death or heart transplant (HT), were 93.6 and 98.5%, respectively. In patients with RP, 10-year freedom-from-death or aSCD, and death or HT, were 59.0 and 71.2%, respectively. RP conferred a 3.5-fold increase in incidence rate of hospitalization (P = 0.01), a 3.8-fold increase in hazard of death or aSCD (P = 0.02), and a 5.7-fold increase in hazard of death or HT (P = 0.04). Assessment for RP is of paramount importance in children with HCM because those without RP have a good prognosis, and those with RP account for the majority of poor outcomes.

    View details for DOI 10.1007/s00246-011-0106-6

    View details for Web of Science ID 000298660100021

    View details for PubMedID 21892651

  • Twenty-Five Year Experience With Balloon Aortic Valvuloplasty for Congenital Aortic Stenosis AMERICAN JOURNAL OF CARDIOLOGY Maskatia, S. A., Ing, F. F., Justino, H., Crystal, M. A., Mullins, C. E., Mattamal, R. J., Smith, E. O., Petit, C. J. 2011; 108 (7): 1024-1028

    Abstract

    Balloon aortic valvuloplasty (BAV) is the primary therapy for congenital aortic stenosis (AS). Few reports describe long-term outcomes. In this study, a retrospective single-institution review was performed of patients who underwent BAV for congenital AS. The following end points were evaluated: moderate or severe aortic insufficiency (AI) by echocardiography, aortic valve replacement, repeat BAV, surgical aortic valvotomy, and transplantation or death. From 1985 to 2009, 272 patients who underwent BAV at ages 1 day to 30.5 years were followed for 5.8 ± 6.7 years. Transplantation or death occurred in 24 patients (9%) and was associated with depressed baseline left ventricular shortening fraction (LVSF) (p = 0.04). Aortic valve replacement occurred in 42 patients (15%) at a median of 3.5 years (interquartile range 75 days to 5.9 years) after BAV and was associated with post-BAV gradient ≥25 mm Hg (p = 0.02), the presence of post-BAV AI (p = 0.03), and below-average baseline LVSF (p = 0.04). AI was found in 83 patients (31%) at a median of 4.8 years (interquartile range 1.4 to 8.7) and was inversely related to post-BAV gradient ≥25 mm Hg (p <0.04). AI was associated with depressed baseline LVSF (p = 0.02). Repeat valvuloplasty (balloon or surgical) occurred in 37 patients (15%) at a median of 0.51 years (interquartile range 0.10 to 5.15) and was associated with neonatal BAV (p <0.01), post-BAV gradient ≥25 mm Hg (p = 0.03), and depressed baseline LVSF (p = 0.05). In conclusion, BAV confers long-term benefits to most patients with congenital AS. Neonates, patients with post-BAV gradients ≥25 mm Hg, and patients with lower baseline LVSF experienced worse outcomes.

    View details for DOI 10.1016/j.amjcard.2011.05.040

    View details for Web of Science ID 000295863200022

    View details for PubMedID 21791328

  • Congenital Anomalies of the Mitral Valve CONGENITAL HEART DISEASE Maskatia, S. A. 2011; 6 (1): 77-82