Survival prospects of pulmonary arterial hypertension associated with congenital heart disease: Insights from the HOPE registry.

Background: Pulmonary arterial hypertension (PAH) is a severe complication among adult patients with congenital heart disease (ACHD). This study presents real-world data on risk stratification, pharmacotherapy and survival rates in PAH-ACHD.
Methods: Data from PAH-ACHD patients were analyzed using The Hellenic Pulmonary Hypertension Registry (HOPE), spanning eight specialized centers between 2015 and 2023. Patients were categorized into low, intermediate, and high-risk groups using the ESC/ERS three-strata model to assess 1-year mortality risk.
Results: A total of 93 PAH-ACHD patients were included (median age 38.5 years, 60.2 % women). Most patients had an atrial septal defect (37.6 %) or a ventricular septal defect (35.5 %), with 11.8 % presenting with complex ACHD. Eisenmenger syndrome was present in 35.5 % of patients. The proportion of low-risk patients nearly doubled from first to last assessment (24.7 % vs. 40.9 %, p = 0.001). Initially, 52.7 % of patients were on PAH monotherapy, with a subsequent shift towards combination therapy (73.1 %) during follow-up. Over a median follow-up of 5.9 years, 29 patients (31.1 %) died, with 1- and 5-year survival rates of 95.5 % and 81.9 %, respectively. Compared to the high-risk group, low- and intermediate-risk patients exhibited a 70 % and 50 % lower hazard of death, respectively. The lowest survival rates were observed in patients with Eisenmenger physiology.
Conclusion: Survival prospects were favorable for the non-high risk patients in this nationwide cohort of PAH-ACHD patients. The observed shift towards combination therapy use may have contributed to the improvement in 1-year ESC mortality risk, underscoring the importance of timely combination therapy with PAH drugs.
Competing Interests: Declaration of competing interest Dr. A. Arvanitaki has received travel grants from MSD. Dr. Eftychia Demerouti received honorarium and consultation fees from Actelion Pharmaceuticals-Janssen Hellas, MSD Merck Hellas, Elpen and Galenica. Dr. A. Frogoudaki has received travel grants from Boehringer, ELPEN, Genesis Pharma and Pfizer. Dr. I. Tsangaris has received fees for lectures and/or consultations from Actelion, Bayer, ELPEN, GSK, Janssen, MSD, Pfizer and United Therapeutics. Dr. K. Naka reports lecture fees from Amgen, Astra, Bayer, BMS, Boehringer Ingelheim, Janssen, Merck, Novartis, Novo Nordisk, Pfizer, Sanofi, Servier. Athanasios Manginas reports consultation fees from Astra Zeneca, Bayer, Elpen, Janssen, MSD and Novartis. Dr. Giannakoulas has received fees for lectures and/or consultations from Actelion/Janssen, Bayer, Boehringer Ingelheim, ELPEN Pharmaceuticals, Ferrer/Galenica, GlaxoSmithKline, Gossamer-Bio, Merck Sharp and Dohme, Pfizer, Lilly, and United Therapeutics. The rest of the authors report no conflicts of interest.
(Copyright © 2024. Published by Elsevier B.V.)