Echocardiographic Assessment after Palliative Surgery for Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is a clinical term used to describe those forms of congenital heart disease characterized by severe aortic outflow obstruction associated with various degrees of hypoplasia of the mitral valve, left ventricle, ascending aorta, and aortic arch. At Children’s Hospital in Philadelphia, 43 patients underwent palliative surgery for HLHS between January 1984 and March 28, 1985. In the previous 5 years, the 87 children with HLHS diagnosed by echocardiogram or catheterization did not survive beyond 6 weeks. This study was designed to evaluate the survivors of surgery by cardiac ultrasound to establish both a standardized criteria of cardiac function and possible sites of obstruction.