Percutaneous atrial septal defect closure in patients with pulmonary arterial hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a dreaded, although rare, complication of uncorrected atrial septal defect (ASD), one of the most common congenital heart diseases [1]. Most patients with ASD undergo early transcatheter closure, which is the reference standard treatment of ASD and prevents PAH development. The optimal management of patients with ASD and PAH is controversial [2, 3]. When the PAH is severe, surgical closure has produced poor outcomes [4-6], whereas transcatheter closure after lung transplantation (transplant-and-repair strategy) has been found beneficial [7]. The 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines stipulate that ASD closure should be considered only when pulmonary vascular resistance (PVR) is below 5 Wood units (WU) [3]. However, transcatheter shunt closure seems possible in selected patients with higher PVR values, particularly after PAH-specific treatment (treat-and-repair strategy), although the data come only from small cohorts and current recommendations rely primarily on expert opinion [6, 8-10]. Prospective studies are needed to further assess this strategy. In the present study, we aimed to assess short-term and long-term outcomes of patients managed by transcatheter ASD closure for persistent left-to-right shunting with PAH.