The Norwood Operation in Infants with Complex Congenital Heart Disease

Park, Jeong Jun; Kim, Yong Jin

Abstract

From April 1987 to May 1996, 13 infants underwent a Norwood operation for complex congenital heart diseases including hypoplastic left heart syndrome(n=7), mitral stenosis with small VSD and subaortic stenosis(n=1), mitral atresia with ventricular septal defect, coarctation of aorta, and subaortic stenosis(n=1), interrupted aortic arch with ventricular septal defect and subaortic stenosis(n=1), tricuspid atresia with transposition of the great arteries(n=1), and complex double-inlet left ventricle(n=2). All patients without hypoplastic left heart syndrome were associated with hypoplasia of ascending aorta and arch. Age at operation ranged from 3 days to 8.7 months(mean 60.6+/-71.6 days, median 39 days). The operative mortality(<30 days) was 46%(6 patients). Late mortality was 15%(2 patients). All operative deaths occured during the first 24 hours after the operation as a result of cardiopulmonary bypass weaning failure(5 patients) and sudden hemodynamic instability postoperatively(1 patient). Late death was due to aspiration pneumonia in two cases. There are 5 long-term survivals(39%). Three of them have undergone a two-stage repair with a modified Fontan operation in two and total cavopulmonary shunt in one at 12, 17, 4.5 months after Norwood procedure with no mortality. Two patients have entered a three-stage repair strategy by undergoing a bidirectional cavopulmonary shunt at 3 and 5.5 months after initial operation with 1 operative death. The actuarial survival rate for all patients at the first-stage operation, including hospital deaths and late death was 30.8% at 1 year. In conclusion, the operative mortality of Norwood operation was relatively high compared to other operation for major cardiac anomalies, continuing experience will lead to an improvement in result.