Definitive operation for pulmonary atresia with intact ventricular septum. Results in twenty patients.

Definitive operation was performed on 20 patients (aged 25 to 178 months) with pulmonary atresia and intact ventricular septum. All patients had one or more prior preliminary palliative procedures. Right ventricular outflow tract reconstruction with atrial septal defect closure and shunt removal was done on 10 patients. Tricuspid annular circumference was at least 70% of normal in seven patients and between 55% and 70% in three patients. Two patients died during hospitalization. The eight surviving patients were asymptomatic 3 to 145 months after operation. The modified Fontan operation was performed on 10 patients. None of these patients had a tricuspid annular circumference greater than 70% of normal; the circumference was less than 55% in nine patients and between 55% and 70% in one patient. One patient died during hospitalization and one died later. Follow-up 6 to 48 months after operation showed that six patients were in the New York Heart Association Class I and two were in Class II. After effective preliminary palliation of pulmonary atresia with intact ventricular septum, definitive operation can be done with an operative risk of 15% (three of 20 patients) and excellent late results. Right ventricular outflow tract reconstruction can be done as a complete repair for patients who have adequate tricuspid annular size. The modified Fontan operation is the only option for definitive repair when the tricuspid anulus is severely hypoplastic.