Lung Transplantation in Pulmonary Arterial Hypertension: A Review

Lung transplantation remains a key treatment option for patients with pulmonary arterial hypertension (PAH) who continue to deteriorate despite targeted medical therapy. However, lung transplant remains underutilized in PAH, and these patients have among the highest waitlist mortality of any transplant indication. This narrative review, based on a PubMed search and author selection of the literature, synthesizes evidence on indications for lung transplant referral in PAH, risk stratification scores that provide insight into disease status, and the evolving medical therapies for PAH, including sotatercept, and their potential impact on referral and transplant practice. We then review preoperative and perioperative strategies for PAH patients, including hemodynamic support, primary graft dysfunction, extracorporeal membrane oxygenation, and the choice between bilateral lung and heart–lung transplantation, along with post-transplant outcomes. We also review geographic and institutional differences in organ availability and outcomes that may affect patients with PAH. Our findings suggest a need for earlier referral, research on how newer medications will affect referral and outcomes, and standardization of surgical and systemic practices to achieve good outcomes in this unique population.