RESULTS OF NORWOOD OPERATION FOR LESIONS OTHER THAN HYPOPLASTIC LEFT-HEART SYNDROME

Norwood's operation provides satisfactory palliation for neonates with hypoplastic left heart syndrome, The dominant physiologic features of hypoplastic left heart syndrome, ductal dependency of the systemic ci rculation and parallel pulmonary and systemic circulations, are shared by a multitude of other less common congenital heart malformations. T heoretically, these should be equally amenable to palliation by Norwoo d's operation, Between January 1990 and June 1994, 60 neonates with ma lformations other than hypoplastic left heart syndrome underwent initi al surgical palliation by Norwood's procedure. Diagnoses included sing le left ventricle with levo-transposition of the great arteries (12); critical aortic stenosis (8); complex double-outlet right ventricle (8 ); interrupted aortic arch with ventricular septal defect and subaorti c stenosis (7); ventricular septal defect, subaortic stenosis, and coa rctation of the aorta (7); aortic atresia with large ventricular septa l defect (6); tricuspid atresia with transposition of the great arteri es (6); heterotaxy syndrome with subaortic obstruction (3); and other (3), There were 10 hospital deaths and 50 survivors (83% survival), Af ter the introduction of inspired carbon dioxide therapy into the posto perative management protocol (1991), 42 of 47 patients survived (89% s urvival), Mortality was independent of diagnosis and essentially the s ame as that for hypoplastic left heart syndrome. With minor technical modifications, Norwood's operation provides satisfactory initial palli ation for a wide variety of malformations characterized by ductal depe ndency of the systemic circulation in anticipation of either a Fontan procedure or a biventricular repair.