Pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals: neonatal pulmonary artery rehabilitation without unifocalization

Abstract

Background: This study analyzed a protocol of neonatal rehabilitation of hypoplastic pulmonary arteries in the management of pulmonary atresia, ventricular septal defect (VSD), and major aortopulmonary collateral arteries (MAPCAs). Ideal management of patients with pulmonary atresia, VSD, and MAPCAs is the subject of controversy.

Methods: From June 2003 to December 2008, 25 consecutive patients were diagnosed with pulmonary atresia, VSD, and MAPCAs, and 20 were entered into a neonatal shunting regimen. The median age at the first operation was 3.6 weeks (range, 0.7 to 17 weeks). All patients underwent an initial central or modified Blalock-Taussig shunt, or both. Further preparatory procedures included 26 pulmonary artery patch reconstructions, 19 right ventricle-to-pulmonary artery conduits, 4 MAPCA ligations, and 4 further shunts. No patient underwent translocation of the collateral arteries.

Results: At the latest follow-up, 12 of 20 patients have had a complete repair at a median age of 18 months (range, 11 to 48 months), 6 are awaiting repair, and 2 are considered unlikely to be repaired. No patient was missing follow-up. Median pulmonary artery indices had grown from 14.51 to 118.7 in 17 patients. Postoperative angiograms were performed at a median of 8 months (range, 1.9 to 32.7 months) in 9 of 12 completely repaired patients. The median right/left ventricular pressure ratio was 0.64 (range, 0.54 to 0.91).

Conclusions: Rehabilitation of hypoplastic native pulmonary arteries by a neonatal shunting regimen, without MAPCA translocation, for pulmonary atresia, VSD, and MAPCAs, provides encouraging results with excellent early survival.