Surgical unifocalization in pulmonary atresia and ventricular septal defect. A realistic goal?

Between 1979 and 1986, 26 patients with pulmonary atresia, ventricular septal defect, and multifocal pulmonary blood supply underwent surgery (median age, 2 years) to reconnect major aortopulmonary collateral arteries to other collateral arteries or to the central pulmonary arteries in an attempt to prepare the pulmonary circulation for complete repair. In four patients, central pulmonary arteries were found to be absent at exploratory thoracotomy, and therefore, the surgery aimed for optimal palliation. Four (15%) patients died perioperatively. The survivors had symptomatic improvement. At restudy 1 week-110 months (median time, 25 months) postoperatively, modest growth of central pulmonary arteries had occurred [right plus left pulmonary artery diameters/descending aortic diameter from 0.64 +/- 0.61 (mean +/- SD) preoperatively to 0.95 +/- 0.60 postoperatively (p less than 0.01)], with a greater number of bronchopulmonary segments connected to central pulmonary arteries [from 6.3 +/- 4.8 preoperatively to 10.0 +/- 4.5 postoperatively (p less than 0.001)]. However, only seven (27%) patients met the established criteria for repair, of whom three (12%) have undergone definitive surgery. Thirteen (50%) patients are unsuitable for repair, and two (8%) await restudy. Failure of anastomoses involving major aortopulmonary collateral arteries (13 of 33) was higher than for grafts from a systemic artery to the central pulmonary artery (1 of 22, p = 0.003). Failure was not related to the number of anastomoses per operation, suggesting that poor distal runoff may have been responsible. This surgical approach provided reasonable palliation but was rarely effective as a prelude to complete repair.(ABSTRACT TRUNCATED AT 250 WORDS)