Physiologic Repair of Aortic Atresia–Hypoplastic Left Heart Syndrome

Abstract

AORTIC-VALVE atresia associated with mitral atresia or stenosis, a diminutive or absent left ventricle, and severe hypoplasia of the ascending aorta and aortic arch make up a constellation of defects that was initially termed "hypoplasia of the aortic tract complexes" by Lev1 and later called "hypoplastic left heart syndrome" by Noonan and Nadas.2 The anatomic complexity of this cardiac malformation has been considered prohibitive of long survival, and generally, only supportive therapy has been recommended. Indeed, aortic atresia–hypoplastic left heart syndrome is uniformly fatal without surgical intervention, and prolonged survival after reparative surgery has not been reported. It is, however, . . .