First-stage palliation of complex univentricular cardiac anomalies in older infants

Abstract

Background. Poor outcomes have been reported for children older than 30 days of age with cardiac anomalies treated with first-stage palliation. Methods. Our institution has offered first-stage palliation for all such patients regardless of age. The results of this policy were reviewed. Results. Nine patients older than 30 days (median age 67 days, range 36 to 108 days) with diagnoses of hypoplastic left heart syndrome (n = 5), double-outlet right ventricle with hypoplastic aortic arch (n = 2), unbalanced atrioventricular septal defect (n = 1), or single left ventricle with subaortic stenosis (n = 1) underwent surgical palliation. Patients underwent a Norwood (n = 7) or Damus-Kaye-Stancel (n = 2) procedure with a 4- or 5-mm modified Blalock-Taussig shunt; all patients survived the operation. Eight patients underwent a subsequent bidirectional Glenn (2 perioperative deaths, both due to pneumonia; 6 survivors). Two of the 6 surviving patients have undergone Fontan reconstruction and 4 are awaiting Fontan. Conclusions. Surgical palliation for complex univentricular cardiac malformations can be performed in older infants with results comparable to those in neonates. The use of a larger shunt may contribute to these improved outcomes.