Aortic atresia with interrupted aortic arch: reparative operation.

Aortic atresia is a form of congenital cardiac disease always complicated by associated severe hypoplasia of the ascending aorta and various degrees of mitral valve and left ventricular hypoplasia. Rarely, there is an associated ventricular septal defect (VSD) and, consequently, a well-developed left ventricle. This complex malformation is universally lethal and survived after a reparative operation has not been previously reported. The following is an account of an infant with aortic atresia, VSD, and Type C aortic arch interruption who presented with a unique ductus-dependent physiology and is clinically well 11 months following a reparative operation.