Shiraz A. Maskatia

Twenty-Five Year Experience With Balloon Aortic Valvuloplasty for Congenital Aortic Stenosis

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.

Aortic valve morphology is associated with outcomes following balloon valvuloplasty for congenital aortic stenosis.

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.

Effect of Branch Pulmonary Artery Stenosis on Right Ventricular Volume Overload in Patients With Tetralogy of Fallot After Initial Surgical Repair

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.

Impact of Obesity on Ventricular Size and Function in Children, Adolescents and Adults With Tetralogy of Fallot After Initial Repair

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.

Repeat balloon aortic valvuloplasty effectively delays surgical intervention in children with recurrent aortic stenosis

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.

Pilot study of chronic maternal hyperoxygenation and effect on aortic and mitral valve annular dimensions in fetuses with left heart hypoplasia.

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.

Congenital heart anomaly in newborns with congenital diaphragmatic hernia: a single-center experience.

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).

Hypertrophic Cardiomyopathy: Infants, Children, and Adolescents

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.

Fetal left‐sided cardiac structural dimensions in left‐sided congenital diaphragmatic hernia – association with severity and impact on postnatal outcomes

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.

Older Age at Completion of Fontan Procedure Is Associated with Improved Percentage of Predicted Maximum Oxygen Uptake

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.