Double-lung transplantation followed by delayed percutaneous repair for atrial septal defect-associated pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a rare but severe complication of unrepaired atrial septal defect (ASD) and appears to be among the strongest predictors of death in adults with Eisenmenger syndrome [1]. For decades, heart-lung transplantation (HLTx) has been considered the best treatment of last resort for patients with ASD-associated PAH and right ventricular failure. Nonetheless, over 25% of patients die within 1 year after the procedure [2]. In addition, the severe organ shortage results in long waitlist times, during which the frequency of clinical deterioration or death exceeds 30% [3]. Double-lung transplantation (DLTx) with concomitant surgical cardiac-defect repair has been suggested as an attractive alternative for patients with ASD-associated PAH [4]. However, due to the limited number of patients with ASD-associated PAH treated by HLTx or DLTx, the available scientific evidence is insufficient to define the best strategy. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: None for any authors