Hybrid Approach in Hypoplastic Left Heart Syndrome (HLHS)

Abstract

Hypoplastic left heart syndrome is a rare congenital heart defect in which the left side of the heart is underdeveloped and the right ventricle supporting both systemic and pulmonary circulation. HLHS is accounting for 2–3 % of all congenital heart defects. Untreated, HLHS is a fatal congenital heart defect. HLHS is morphologically subdivided in mitral and/or aortic atresia or stenosis, respectively. About 6 % of HLHS patients have a completely intact atrial septum at birth and up to 22 % a severely restrictive atrial septum. Intact or highly restrictive atrial septum (IAS) is associated with a high rate of mortality. Another form of pulmonary venous flow obstruction might be associated with total anomalous pulmonary venous return (TAPVR). Neonates that present with hypoplastic left heart syndrome (HLHS) are clinically stable as long as the parallel circulation is balanced.