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Atrial Flow Regulator: Bridging Pediatric Patients With Pulmonary Arterial Hypertension to Lung Transplantation
Julia Moosmann1, Anastasia Schleiger1, Felix Berger2
1Department of Congenital Heart Disease-Pediatric Cardiology, Deutsches Herzzentrum der Charité (DHZC), Berlin, Germany; Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Insights
Atrial flow regulators (AFR) help stabilize pediatric patients with severe pulmonary arterial hypertension (PAH) and right heart failure, serving as a bridge to lung transplantation (LTX). Post-transplant closure of the AFR minimizes long-term risks.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension Research
- Cardiothoracic Surgery
Background:
- Pediatric pulmonary arterial hypertension (PAH) with right heart failure is challenging, especially when medical therapy fails.
- An atrial flow regulator (AFR) offers a novel approach for right ventricular decompression and bridges patients to lung transplantation (LTX).
- Limited data exists on pre-LTX decisions, perioperative care, and post-LTX management in pediatric PAH patients.
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.
