Fifteen-year single-center experience with the Norwood operation for complex lesions with single-ventricle physiology compared with hypoplastic left heart syndrome

Abstract

The Norwood procedure, the first surgical step of staged palliation for hypoplastic left heart syndrome, is also applied for other complex single-ventricle lesions with systemic outflow tract obstruction or aortic arch hypoplasia. We reviewed our 15-year institutional experience with the Norwood procedure for patients with and without hypoplastic left heart syndrome. A total of 41 patients without hypoplastic left heart syndrome and 212 patients with hypoplastic left heart syndrome who underwent a Norwood procedure between January 1996 and December 2010 were enrolled. Full medical records were reviewed to assess the determinants of outcome. Early failure (death or cardiac transplantation) was 7% in patients without hypoplastic left heart syndrome and 13% in patients with hypoplastic left heart syndrome (P = .29). Frequency of postoperative complications, duration of postoperative ventilation, and length of vasoactive drug treatment were not different between groups. Transplant-free survival until the second operative step trended to be higher for patients without hypoplastic left heart syndrome (92% vs 80%, P = .067). Recurrent aortic arch obstruction was more common in patients without hypoplastic left heart syndrome (15/39 vs 32/171, P = .008), but there were 4 patients with stenosis of the proximal aortic arch. In subsequent procedures, 31 patients without hypoplastic left heart syndrome underwent superior cavopulmonary anastomosis and 5 biventricular repair. Overall transplant-free survival was not different between groups (P = .119) but trended to be higher in patients with a systemic or substantial left ventricle remnant contributing to cardiac output (P = .082). Early and long-term survivals and postoperative complications were similar between patients with and without hypoplastic left heart syndrome undergoing a Norwood operation. Recurrent aortic arch obstruction was common in both groups but more prevalent in patients without hypoplastic left heart syndrome.