Julia Moosmann, Anastasia Schleiger, Felix Berger, Peter Kramer
Deutsches Herzzentrum der Charité (DHZC), Berlin, Charité-Universitätsmedizin Berlin, Freie Universität Berlin and Humboldt-Universität zu Berlin.
Germany
Journal of the American College of Cardiology Case Reports
JACC Case Rep 2025;
DOI: 10.1016/j.jaccas.2025.106118
Abstract
Background: Pulmonary arterial hypertension (PAH) in pediatric patients remains challenging, particularly in symptomatic patients with right heart failure despite optimal medical therapy. The use of an atrial flow regulator (AFR) to decompress the right ventricle has offered a new therapeutic approach for those patients and allows bridging for lung transplantation (LTX). Data on pretransplant decision-making, perioperative considerations, and post-transplant management in pediatric patients undergoing LTX are limited.
Case presentation: We report 2 pediatric patients with severe symptomatic PAH and right heart failure in whom an AFR was implanted before LTX. In both patients, AFR implantation resulted in clinical stabilization, allowing for successful listing and subsequent LTX. After LTX, both patients showed complete cardiac remodeling, and the AFR fenestration was closed percutaneously to minimize long-term complications.
Conclusions: AFR implantation is a valuable bridging strategy to LTX in pediatric patients with therapy-refractory PAH, enabling clinical stabilization and safer perioperative management. Post-transplant transcatheter closure is reasonable to reduce long-term risks associated with persistent atrial shunting.
Category
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Lung Transplantation for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes