Combined Double-Lung Transplantation and Percutaneous Septal Defect Closure for End-Stage Atrial Septal Defect-Associated Pulmonary Arterial Hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a rare but severe complication of unrepaired atrial septal defect (ASD) and carries a worse prognosis than posttricuspid shunt. In patients with ASD complicated by PAH and right ventricular failure, heart-lung transplantation (HLT) is currently viewed as the best option despite high postoperative mortality. However, the severe donor organ shortage results in high waiting-list mortality. Our objective here was to compare the safety and benefits of double-lung transplantation (DLT) followed by percutaneous ASD closure versus HLT for end-stage ASD-associated PAH. We retrospectively reviewed patients with end-stage ASD-associated PAH treated by DLT plus percutaneous ASD closure or HLT at our center. Posttransplant right heart catheterization was performed before ASD closure to ensure the absence of residual significant PAH. Of 16 included patients, 10 received HLT and 6 DLT followed by percutaneous ASD closure after a median of 4.3 months. Demographics and preoperative clinical and hemodynamic severity parameters were comparable between groups. No between-group differences were found for primary graft dysfunction, ventilation duration, dialysis in the ICU, or 3-month mortality. Survival rates at 1, 3, and 5 years were 100%, 80%, and 80% after DLT plus percutaneous ASD closure and 60%, 60%, and 60% after HLT. DLT plus percutaneous ASD closure is safe and provides outcomes that compare favorably to those of HLT in patients with end-stage ASD-associated PAH.