Comparison of Necrotizing Enterocolitis After Surgical Norwood versus Hybrid Stage 1 Palliation.

Hybrid stage 1 palliation (HS1P) is used as an alternative to the surgical Norwood for single ventricle patients and specific other conditions. Necrotizing enterocolitis (NEC) occurs in 5%-18% of infants after surgical Norwood, contributing to significant morbidity. We sought to compare the NEC incidences in HS1P versus Norwood patients.

Early and Midterm Outcomes in High-risk Single-ventricle Patients: Hybrid Vs Norwood Palliation.

Background: Although overall outcomes have improved for single-ventricle patients, substantial morbidity and mortality remain for certain high-risk groups. The hybrid stage I procedure is an alternative to the Norwood operation for stage I palliation, but it remains unclear whether it is associated with improved outcomes in high-risk patients.

Methods: This single-center, nested, case-control study included high-risk patients with a systemic right ventricle who underwent hybrid stage I or Norwood palliation from January 2000 to December 2016.

Pilot study analyzing automated ECG screening of hypertrophic cardiomyopathy.

Background: Hypertrophic cardiomyopathy (HCM) is one of the leading causes of sudden cardiac death in athletes. However, preparticipation ECG screening has often been criticized for failing to meet cost-effectiveness thresholds, in part because of high false-positive rates and the cost of ECG screening itself.

Objective: The purpose of this study was to assess the testing characteristics of an automated ECG algorithm designed to screen for HCM in a multi-institutional pediatric cohort.

The application of root mean square electrocardiography (RMS ECG) for the detection of acquired and congenital long QT syndrome.

Background: Precise measurement of the QT interval is often hampered by difficulty determining the end of the low amplitude T wave. Root mean square electrocardiography (RMS ECG) provides a novel alternative measure of ventricular repolarization. Experimental data have shown that the interval between the RMS ECG QRS and T wave peaks (RTPK) closely reflects the mean ventricular action potential duration while the RMS T wave width (TW) tracks the dispersion of repolarization timing.

Stentless porcine valves in the right ventricular outflow tract: improved durability?

Objective: Stentless porcine valves are commonly used for aortic valve replacement in adults, yet their long-term performance in the right ventricular (RV) outflow tract is unknown. We evaluated intermediate-term performance of stentless porcine valves in the RV outflow tract in 150 children and adults over a 10-year period.

Methods: We retrospectively reviewed data on all patients undergoing placement of a pulmonary valve or RV-PA conduit with a stentless porcine prosthesis (>/=19 mm) from 1998 to 2008.

Absence of TGFBR1 and TGFBR2 mutations in patients with bicuspid aortic valve and aortic dilation.

Mutations in the genes encoding transforming growth factor-beta receptor types I and II (TGFBR1 and TGFBR2, respectively) are commonly identified in patients with Loeys-Dietz syndrome, as well as some patients with Marfan's syndrome or familial thoracic aortic aneurysms and dissections. This suggests that there is considerable phenotypic heterogeneity associated with mutations in these genes. Because bicuspid aortic valve (BAV) is a congenital heart defect in patients with Loeys-Dietz syndrome, this study was conducted to investigate whether variants in TGFBR1 or TGFBR2 are responsible for sporadic BAV.

Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy?

Background: Progressive cardiomyopathy is a common cause of death in Duchenne muscular dystrophy (DMD), presumably secondary to fibrosis of the myocardium. The posterobasal and left lateral free wall of the left ventricle (LV) are initial sites of myocardial fibrosis pathologically. The purposes of this study were to assess whether cardiac magnetic resonance imaging (CMRI), utilizing late gadolinium enhancement (LGE), could identify fibrosis in selective areas of the myocardium, and to assess the relationship of the presence and extent of fibrosis to LV function.

Pulmonary regurgitation: determining severity by echocardiography and magnetic resonance imaging.

Objective: Pulmonary regurgitation (PR) is common after repair of congenital heart disease involving the right ventricular outflow tract. Because PR results in chronic right ventricular volume overload and associated morbidity and mortality, accurate assessment of its severity is important. The aim of this study was to compare echocardiography with the gold standard of PR quantitation by magnetic resonance imaging (MRI) in a young population with repaired congenital heart disease.

Right ventricle-to-pulmonary artery conduit longevity: is it related to allograft size?

Background: Cryopreserved valved allografts are routinely oversized to account for somatic growth in children requiring right ventricle-to-pulmonary artery (RV-PA) continuity. The objective of this study is to determine the effect of oversizing on conduit longevity.

Methods: We reviewed the records of all patients undergoing RV-PA cryopreserved valved allograft placement from 1988 to 2006 for diagnosis, age, allograft type, time to valved conduit explant, and indication for surgery.