A male newborn infant with hypoplastic left heart and transposition of the great arteries died on the second day of life. The haemodynamic consequences of this malformation are considerably different from those of ordinary hypoplastic left heart syndrome because of pulmonary rather than aortic atresia. In the absence of cross sectional echocardiographic equipment, cardiac catheterisation with a...

Hypoplastic left heart syndrome (HLHS) is the most common functional single ventricle congenital cardiac defect. This syndrome is characterised by a functional single right ventricle and systemic outflow obstruction. The systemic and pulmonary circulations compete for cardiac output with a resultant precarious balance among systemic, pulmonary and coronary blood flows. A once fatal diagnosis, a...

Hypoplastic left heart syndrome (HLHS) is one of the most challenging congenital heart defects. At present, it is expected that - at best - 70% of newborns with HLHS will reach adulthood. This review addresses the problems of right ventricular (RV) failure and insufficient growth of pulmonary vasculature in these patients. In order to further improve long-term prognosis translational research t...

serum. A review of the reports on similar cases showed no male preponderance (50%). In contrast, the heterogeneous group ofhypoplastic left heart syndrome showed 64per cent male preponderance. The mean maternal age was 3I years in hypoplastic left heart syndrome, excluding patients with interruption of the aortic arch and mitral and aortic valve atresia where the mean maternal age was lower, 22...

OBJECTIVES The study objectives were to compare the neurodevelopmental outcomes of preschool survivors of the Fontan procedure with those of children with congenital heart disease undergoing biventricular repair and to investigate predictors of neurodevelopmental outcome for those with single ventricle congenital heart disease, including hypoplastic left heart syndrome. METHODS Neurodevelopme...

OBJECTIVES This study sought to determine the size of the genetic effect (heritability) in families identified by a hypoplastic left heart syndrome (HLHS) proband. BACKGROUND Hypoplastic left heart syndrome is a severe form of cardiovascular malformation (CVM), and it remains a leading cause of infant mortality and childhood morbidity. Familial clustering of HLHS and bicuspid aortic valve (BA...

PURPOSE Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. METHODS A retrospective analysis was ...