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

Extract

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.