Adverse Effect of Bundle Branch Block on Exercise Performance in Patients with Fontan Physiology: From the Pediatric Heart Network Fontan Public Data Set.

Patients with Fontan physiology have reduced exercise performance compared to their peers as well as a higher incidence of bundle branch block (BBB). This study aims to investigate the association between BBB and exercise performance in the Fontan population through a retrospective review of the Pediatric Heart Network Fontan study public use dataset. "Low Performers" were defined as ≤ 25th percentile (for Fontan patients) for each exercise parameter at anaerobic threshold (AT) for gender and age and "Normal Performers" were all other patients.

Worse Hospital Outcomes for Children and Adults with COVID-19 and Congenital Heart Disease.

The aim of the current study is to investigate hospitalization outcomes of COVID-19 positive children and adults with moderate or severe congenital heart disease to children and adults without congenital heart disease. Retrospective review using the Vizient Clinical Data Base for admissions of patients with an ICD-10 code for COVID-19 from April 2020 to March 2021. Admissions with COVID-19 and with and without moderate or severe congenital heart disease (CHD) were stratified into pediatric (< 18 years) and adult (≥ 18 years) and hospital outcomes were compared.

Exercise Performance in Adolescents With Fontan Physiology (from the Pediatric Heart Network Fontan Public Data Set).

In the pediatric population, exercise capacity differs between females and males and the gap widens through adolescence. However, specific age- and sex-based changes in adolescents with congenital heart disease and Fontan palliation have not been reported. The purpose of the current study is to identify age- and sex-specific changes in exercise performance at peak and ventilatory anaerobic threshold (AT) for adolescents with Fontan physiology.

Development of bilateral coronary artery aneurysms in a child with Noonan syndrome.

Noonan syndrome is a constellation of congenital malformations including heart defects, facial anomalies and short stature. The cardiovascular defects are variable and extensive, with the most common being pulmonary stenosis and hypertrophic cardiomyopathy. Coronary artery anomalies have only been reported in a few cases.

Cardiac function in congenital adrenal hyperplasia: a pattern of reversible cardiomyopathy.

Objective: To evaluate cardiac function in infants with congenital adrenal hyperplasia (CAH) before and after corticosteroid replacement therapy.

Study Design: This prospective, case-control study included 9 infants with CAH. Cardiac function was assessed by echocardiography at presentation and after corticosteroid replacement therapy.

Pulmonary vein atresia with severe contralateral pulmonary vein stenosis in a child.

Unilateral pulmonary vein atresia is a very rare congenital anomaly associated with high morbidity and mortality. Most cases present in infancy or childhood with recurrent respiratory infections or hemoptysis. Further, the diagnosis can often be difficult to make.

Assessment of the accuracy and reproducibility of RV volume measurements by CMR in congenital heart disease.

Objectives: The purpose of this study was to determine whether right ventricular (RV) volumes are more accurately and reproducibly measured by cardiac magnetic resonance (CMR) in an axial orientation or in a short-axis orientation in patients with congenital heart disease (CHD).

Background: There is little agreement on the most suitable imaging plane for RV volumetric analysis in the setting of abnormal RV physiology.

Methods: Measurements of RV volumes from datasets acquired in axial and short-axis orientations were made in 50 patients with CHD.

Anatomically corrected malposition of the great arteries.

We present cardiac magnetic resonance images of a 6-year-old with a suspected great artery abnormality in the setting of mesocardia, atrial situs solitus, D-looping of the ventricles, and anatomically corrected malposition of the great arteries with subaortic and subpulmonary conus.

Accuracy of plasma B-type natriuretic peptide to diagnose significant cardiovascular disease in children: the Better Not Pout Children! Study.

Objectives: The purpose of this study was to assess the ability of plasma B-type natriuretic peptide (BNP) to diagnose significant cardiovascular disease (CVD) in the pediatric population.

Background: BNP has been shown to be reliable in detecting ventricular dysfunction and heart failure in adults. Timely and accurate identification of significant pediatric heart disease is important but challenging.

The safety and efficacy of ibutilide in children and in patients with congenital heart disease.

Background: The safety and efficacy of ibutilide in the cardioversion of atrial flutter and atrial fibrillation in children and in patients with congenital heart disease (CHD) is unknown.

Methods: Data from 19 patients (age 6 months to 34 years, median 16 years) who received ibutilide for atrial flutter or atrial fibrillation between 1996 and 2005 was retrospectively reviewed. There were 15 patients with CHD (14 had prior heart surgery); four children had normal heart structure.

A novel approach incorporating sildenafil in the management of symptomatic neonates with Ebstein's anomaly.

The neonatal presentation of Ebstein's anomaly is a distinct cyanotic lesion for which little can be done other than to maintain ductal patency or perform palliative surgery if improvement does not occur. We report two cases in which sildenafil was introduced to assist previously failed attempts at weaning of prostaglandin.