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

Abstract

Objective: 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.

Methods: 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.

Results: 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).

Conclusions: 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.