TY - JOUR T1 - Double-lung transplantation followed by delayed percutaneous repair for atrial septal defect-associated pulmonary arterial hypertension JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.02388-2021 VL - 59 IS - 1 SP - 2102388 AU - Pierre Gazengel AU - Sébastien Hascoët AU - Myriam Amsallem AU - Laurent Savale AU - David Montani AU - Olaf Mercier AU - Marc Humbert AU - Elie Fadel AU - Jérôme Le Pavec Y1 - 2022/01/01 UR - http://erj.ersjournals.com/content/59/1/2102388.abstract N2 - 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, severe organ shortages result in long waiting list 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.In pulmonary hypertension with right heart failure due to atrial septal defect, double-lung transplantation then percutaneous defect closure 3–6 months later compared favourably with the standard approach consisting of heart–lung transplantation https://bit.ly/3BjdLzw ER -