Transcatheter pulmonary valve replacement in congenital heart diseases.

Surgical repair of a variety of congenital heart diseases involves repair of the right ventricular outflow tract (RVOT) with valved or non-valved conduit to connect the right ventricle (RV) to the pulmonary artery (PA) or just patch enlargement of the native RVOT. With time, this RV-PA conduit will degenerate with deterioration of function, either causing pulmonary stenosis or pulmonary regurgitation. This RVOT dysfunction may result in RV dilation, RV dysfunction, and eventual RV failure and arrhythmias.

Comparing the accuracy of obstetric sonography and fetal echocardiography during pediatric cardiology consultation in the prenatal diagnosis of congenital heart disease.

Aim: The diagnostic accuracy of fetal echocardiogram performed by an obstetrician alone and that performed jointly by an obstetrician and pediatric cardiologist for congenital heart disease were compared.

Materials And Methods: All cases of suspected fetal congenital heart disease (CHD) referred to the Prenatal Diagnostic Clinic at Tsan Yuk Hospital, Hong Kong during 2006-2011 were reviewed. Prenatal fetal echocardiogram findings were compared with postnatal diagnosis.

NGS identifies TAZ mutation in a family with X-linked dilated cardiomyopathy.

We reported a family with two male siblings affected with infantile dilated cardiomyopathy (DCM). Extensive evaluation failed to identify the underlying cause for the DCM. Next generation sequencing (NGS) with targeted enrichment identified a hemizygous variant c.

Brain natriuretic peptide as a biomarker of systemic right ventricular function in patients with transposition of great arteries after atrial switch operation.

Background: Assessment of systemic right ventricular (RV) function is inherently difficult. In adults, plasma brain natriuretic peptide (BNP) level has been shown to reflect systemic ventricular dysfunction. We sought to test the hypothesis that plasma BNP is a biomarker of systemic RV function in patients after atrial switch operation.

Cardiac rhythm and symptomatic arrhythmia in right atrial isomerism.

Background: The conduction system in right atrial isomerism may be complicated by the presence of paired sinus nodes and twin atrioventricular nodes. With a large cohort of infants and children with right atrial isomerism, we sought to investigate the nature of atrial rhythm, its prevalence, factors predisposing patients to symptomatic cardiac arrhythmia, and the relation of arrhythmia to long-term morbidity and mortality.

Methods: Standard 12-lead baseline electrocardiograms were performed in 110 infants and children with right atrial isomerism at a median age of 1 day, and their clinical records were reviewed.