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.
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