Staged operation for pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries. New technique for complete unifocalization.

A new staged operation for total correction of pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries has been developed. In first-stage repair (complete unifocalization), intrapulmonary arteries were unified at the hilum with equine pericardial conduits (intrapulmonary bridges). In case of absent or severely hypoplastic central pulmonary arteries, new central pulmonary arteries were created. Finally, the unifocalization was completed by modified Blalock-Taussig shunts with the ligation of collateral arteries. In second-stage repair, right ventricular-pulmonary arterial continuity was established with a trileaflet pericardial conduit and closure of the ventricular septal defect. From January 1982 through July 1988, 34 patients, whose ages ranged from 1 month to 24 years (mean 6.6 years), underwent first-stage repair with two resultant late deaths (mortality rate 5.9%). Second-stage repair has been completed in 16 patients. There were two early deaths (mortality rate 12%) from bacterial infection. Postoperative right ventricular/left ventricular systolic pressure ratios ranged from 0.36 to 1.0 (mean 0.72). In four patients in whom the ratio was 1.0, the ventricular septal defect had been closed with a perforated patch. By complete unifocalization with the intrapulmonary bridge technique and the creation of new central pulmonary arteries, the majority of patients with major aortopulmonary collateral arteries can have successful repair.