Modified Norwood operation for hypoplastic left heart syndrome

Background. We examined early results in infants with hypoplastic left hear t syndrome undergoing the Norwood operation with perioperative use of inhal ed nitric oxide and application of extracorporeal membrane oxygenation. Methods. Medical records were reviewed retrospectively. Results. Between April 1997 and March 2001, 50 infants underwent a modified Norwood operation for hypoplastic left heart syndrome. Mean age at operati on was 7.5 +/- 5.7 days, and mean weight was 3.1 +/- 0.5 kg. Five infants h ad a delayed operation because of sepsis. The mean diameter of the ascendin g aorta by echocardiography was 3.6 +/- 1.8 mm. Ductal cannulation was used to establish cardiopulmonary bypass in all patients. Mean circulatory arre st time was 39.4 +/- 4.8 minutes. The size of the pulmonary-systemic shunt was 3.0 mm in 6 infants, 3.5 mm in 37, and 4.0 mm in 7. Infants with persis tent hypoxia (partial pressure of oxygen < 30 mm Hg) received nitric oxide after they were weaned from cardiopulmonary bypass. Extracorporeal membrane oxygenation was initiated in 8 infants in the pediatric intensive care uni t primarily for low cardiac output and in 8 in the operating room because o f the inability to separate them from cardiopulmonary bypass. The 30-day mo rtality rate was 22% (11 of 50 patients), and the hospital mortality rate w as 32% (16 of 50 patients). Mean follow-up was 17 months. Ten patients (20% ) underwent stage-two repair, with one operative death. One survivor had a Fontan procedure, and 2 underwent heart transplantation, with one death. Conclusions. Early application of extracorporeal membrane oxygenation for h emodynamic instability and selective use of nitric oxide for persistent hyp oxia in the immediate postoperative period may improve survival of patients with hypoplastic left heart syndrome. Renal failure requiring hemofiltrati on during extracorporeal membrane oxygenation (p < 0.05) and cardiopulmonar y arrest in the pediatric intensive care unit (p < 0.05) were predictors of hospital mortality. (C) 2001 by The Society of Thoracic Surgeons.