Single-stage unifocalization and correction with median sternotomy in complex pulmonary atresia.

Objective: This study assessed the surgical and post-operative outcome of single-stage complete unifocalization and repair procedure in patients with complex pulmonary atresia.

Methods: From 1999 to 2001, we performed complete unifocalization and correction in 10 patients with complex pulmonary atresia. Their ages ranged from 10 months to 17 years. All patients were evaluated with pulmonary angiography and divided into two groups according to the development of native pulmonary arteries. Group I patients had hypoplastic pulmonary arteries and MAPCAs and Group II patients had only MAPCAs without native pulmonary arteries. With median sternotomy, all MAPCAs were prepared and anastomozed to native pulmonary arteries in group I patients or on a pericardial roll in group II patients without using cardiopulmonary bypass. Right ventricle to pulmonary arterial continuity was established with a valved conduit under CPB. VSD was closed in two patients.

Results: Eight patients had complete repair without VSD closure. They were followed periodically with pulmonary angiography. Two patients developed congestive heart failure. One of them was reoperated and VSD was closed. The other patient died because of untractable congestive heart failure. The decision for VSD closure was made in two patients due to suitable pulmonary arterial vascular tree. However, one of them had to be reoperated and VSD patch was removed. This patient died because of sepsis on the postoperative 26th day. We are following the rest of the patients with echocardiography and pulmonary angiography.

Conclusion: Single stage complete unifocalization and repair should be the treatment of choice in patients with complex pulmonary atresia. This procedure provides a significant development in neopulmonary arterial system. However, the accurate criterias for VSD closure are still controversial. After the operation, these patients had to be followed closely with echocardiography and pulmonary angiography because of the absolute risk of congestive heart failure in patients with VSD left open.