Add-on therapy with parenteral prostacyclin analogues in patients with pulmonary arterial hypertension: Insights from the COMPERA registry

Parenteral prostacyclin analogues (PPA) are recommended for pulmonary arterial hypertension (PAH) patients at intermediate-high or high risk, yet supporting evidence remains limited. We retrospectively analyzed pretreated patients with idiopathic/heritable/drug-associated PAH (I/H/D-PAH), connective tissue disease-associated PAH (CTD-PAH), or congenital heart disease-associated PAH (CHD-PAH) from the COMPERA registry who received add-on PPA therapy. Among 495 patients, all pretreated with ≥1 PAH medication, add-on PPA treatment was associated with improvements in 6-minute walk distance, WHO functional class, and NT-proBNP across groups. However, mortality was high: Kaplan-Meier survival estimates at 5 years were 59% in I/H/D-PAH, 59% in CHD-PAH, and 31% in CTD-PAH. Independent predictors of mortality included older age, male sex, CTD, CHD, and intermediate-high or high risk at time of PPA initiation. These findings indicate short-term clinical improvements but high subsequent mortality with add-on PPA therapy in patients with PAH, particularly in those with CTD-PAH, underscoring the need for more effective therapies.

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Publication Details

Journal
The Journal of Heart and Lung Transplantation
Published
2025-10-25
DOI
https://doi.org/10.1016/j.healun.2025.10.019
Primary Topic
Pulmonary Hypertension Research and Treatments
Type
article
Field-Weighted Citation Impact
0.00

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article

Add-on therapy with parenteral prostacyclin analogues in patients with pulmonary arterial hypertension: Insights from the COMPERA registry

Roberto Badagliacca, Ralf Ewert, Marius M. Hoeper, Silvia Ulrich et al.
The Journal of Heart and Lung Transplantation
Pulmonary Hypertension Research and Treatments
article

Add-on therapy with parenteral prostacyclin analogues in patients with pulmonary arterial hypertension: Insights from the COMPERA registry

Roberto Badagliacca, Ralf Ewert, Marius M. Hoeper, Silvia Ulrich, Grzegorz Kopeć, Christine Pausch, Andris Skride, Anton Vonk Noordegraaf, David Pittrow, Laura Scelsi, Elena Pfeuffer-Jovic, Dörte Huscher, Michael Halank, Marion Delcroix, Hans F. E. Klose, Stefan Stadler, Khodr Tello, Hossein Ardeschir Ghofrani, Ekkehard Grünig, Karen M. Olsson
article en

Abstract

Parenteral prostacyclin analogues (PPA) are recommended for pulmonary arterial hypertension (PAH) patients at intermediate-high or high risk, yet supporting evidence remains limited. We retrospectively analyzed pretreated patients with idiopathic/heritable/drug-associated PAH (I/H/D-PAH), connective tissue disease-associated PAH (CTD-PAH), or congenital heart disease-associated PAH (CHD-PAH) from the COMPERA registry who received add-on PPA therapy. Among 495 patients, all pretreated with ≥1 PAH medication, add-on PPA treatment was associated with improvements in 6-minute walk distance, WHO functional class, and NT-proBNP across groups. However, mortality was high: Kaplan-Meier survival estimates at 5 years were 59% in I/H/D-PAH, 59% in CHD-PAH, and 31% in CTD-PAH. Independent predictors of mortality included older age, male sex, CTD, CHD, and intermediate-high or high risk at time of PPA initiation. These findings indicate short-term clinical improvements but high subsequent mortality with add-on PPA therapy in patients with PAH, particularly in those with CTD-PAH, underscoring the need for more effective therapies.

The Journal of Heart and Lung TransplantationVol. 45(2)
Jagiellonian University (PL), Heidelberg University (DE), University of Pavia (IT), Universitätsmedizin Greifswald (DE), University Hospital Heidelberg (DE), University Hospital Regensburg (DE), Medizinische Hochschule Hannover (DE), University Hospital Foundation (CA), Pauls Stradiņš Clinical University Hospital (LV), Zimmer Biomet (Germany) (DE), University Hospital of Zurich (CH), John Paul II Hospital (PL), Medical Mission Institute (DE), Amsterdam Neuroscience (NL), German Center for Lung Research (DE), Universities of Giessen and Marburg Lung Center (DE), GWT-TUD (Germany) (DE), Berlin Institute of Health at Charité - Universitätsmedizin Berlin (DE), Cardio-Pulmonary Institute (DE), University Hospital Carl Gustav Carus (DE), University of Regensburg (DE), Technische Universität Dresden (DE), Sapienza University of Rome (IT), Vrije Universiteit Amsterdam (NL), KU Leuven (BE)
National Science Foundation, Amgen, Eli Lilly and Company, Pfizer, AstraZeneca, GlaxoSmithKline, Sanofi, United Therapeutics Corporation, Acceleron, Bayer HealthCare, Deutsche Forschungsgemeinschaft, Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung, Bundesministerium für Bildung und Forschung, EMDO Stiftung, Lungenliga Schweiz, AOP Orphan, Daiichi Sankyo Europe
Openalex Percentile: Top 56%
Pulmonary Hypertension Research and Treatments
DOIOpen Access PDFOpenAlex
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