Evolving Profile and Outcomes of Patients with Pulmonary Arterial Hypertension Undergoing Lung Transplantation

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Background Despite major advances in targeted therapies, lung transplantation remains a key option for selected patients with end-stage pulmonary arterial hypertension (PAH) but is associated with substantial clinical and organizational challenges. Our aim was to describe the impact of changes in advanced PAH management and referral strategies on the profile of transplanted patients and outcomes. Methods All patients with PAH (except congenital heart disease), who underwent lung or heart–lung transplantation between 2008 and 2023 were retrospectively studied in France. Data were obtained from the National PAH and Transplant Registries. Patient characteristics, transplant strategies, and outcomes were compared between 2008–2015 (period 1) and 2016–2023 (period 2). Results Overall, 241 PAH patients underwent transplantation. The proportion of patients aged ≥50 years increased from 30% to 44% (p=0.04). Time from diagnosis to transplantation tended to increase (39 vs. 46 months, p=0.2). Pre-operative extracorporeal membrane oxygenation (ECMO) as a bridge to transplantation increased from 6% to 14% (p=0.05), while post-operative ECMO use remained stable (35% vs. 36%). Three-month post-transplant survival was 85% (95% CI 78–92%) and 91% (95% CI 86–96%) in periods 1 and 2, respectively. In the PAH population aged <65 years, the ratio of deaths without transplantation to transplantations performed exceeded 5:1, with marked heterogeneity across aetiologies. Conclusion Advances in severe PAH management have enabled transplantation in older and more intensively treated patients without compromising short-term outcomes. However, major disparities in access to transplantation persist across PAH aetiologies, underscoring the persistent challenges in advanced PAH management.