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. 2025 May 6;65(5):2400895.
doi: 10.1183/13993003.00895-2024. Print 2025 May.

Initial therapy in patients with pulmonary arterial hypertension and cardiovascular comorbidities

Athénaïs Boucly  1   2   3 , David Montani  1   2   3 , Fabrice Bauer  4 , Elise Artaud-Macari  5 , Emmanuel Bergot  6 , Clément Boissin  7 , Ari Chaouat  8 , Vincent Cottin  9 , Claire Dauphin  10 , Bruno Degano  11 , Pascal De Groote  12 , Camille du Roure  13 , Nicolas Favrolt  14 , Delphine Horeau-Langlard  15 , Xavier Jaïs  1   2   3 , Mitja Jevnikar  1   2   3 , Thomas Lacoste-Palasset  1   2   3 , François Picard  16 , Grégoire Prévôt  17 , Martine Reynaud-Gaubert  18 , Anne Roche  1   2   3 , Ségolène Turquier  9 , Marc Humbert  1   2   3 , Olivier Sitbon  1   2   3   19 , Laurent Savale  20   2   3   19
Affiliations

Initial therapy in patients with pulmonary arterial hypertension and cardiovascular comorbidities

Athénaïs Boucly et al. Eur Respir J. .

Abstract

Background: European guidelines recommend initial monotherapy in pulmonary arterial hypertension patients with cardiovascular comorbidities based on the limited evidence for combination therapy in this growing population.

Methods: A retrospective analysis was conducted on incident pulmonary arterial hypertension patients enrolled in the French Pulmonary Hypertension Registry between 2009 and 2020. Propensity score matching was used to investigate initial dual oral combination therapy versus oral monotherapy in patients with at least one cardiovascular comorbidity (i.e. hypertension, obesity, diabetes and coronary artery disease).

Results: Of the 1784 patients identified, 1088 had at least one cardiovascular comorbidity, including 20% with three or comorbidities. In the propensity score-matched population (n=708), the majority of patients were female, with idiopathic/heritable/drug-induced pulmonary arterial hypertension at intermediate 1-year mortality risk. At first follow-up, initial dual therapy led to larger improvements in symptoms, exercise capacity, haemodynamic parameters and risk status than initial monotherapy, with no differences in long‑term survival. Treatment discontinuation was observed in 23% of patients initiated on dual therapy and 24% of those initiated on monotherapy.

Conclusions: Initial dual oral combination therapy may be beneficial and well-tolerated in most pulmonary arterial hypertension patients with cardiovascular comorbidities.

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Conflict of interest statement

Conflict of interest: A. Boucly was supported by ERS/EU RESPIRE4 Marie Skłodowska-Curie Postdoctoral Research Fellowship (R4202205-00947) and reports grants from Acceleron, Janssen and MSD; payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen, Merck, AOP Orphan, Ferrer and AstraZeneca; and support for attending meetings from Janssen, MSD, Ferrer and AOP Orphan. D. Montani reports grants from Acceleron, Merck MSD and Janssen; consultancy fees from Acceleron, Merck MSD, Janssen and Ferrer; and payment or honoraria for lectures, presentations, manuscript writing or educational events from Bayer, Janssen, Boehringer, Chiesi, GSK, Ferrer and Merck MSD. C. Boissin reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Ferrer and Actelion and support for attending meetings from Janssen. A. Chaouat reports support for attending meetings from MSD and Janssen. V. Cottin reports consulting fees from Ferrer/United Therapeutics and Liquidia, and payment or honoraria for lectures, presentations, manuscript writing or educational events from Ferrer/United Therapeutics. B. Degano reports support for attending meetings from Janssen. C. du Roure (D&A Scientific Services GmbH) provided medical writing support, and reports grants or contracts from Actelion Pharmaceuticals, a Janssen Pharmaceutical Company of Johnson & Johnson, and stock or stock options with Johnson & Johnson. X. Jaïs reports grants from Acceleron, Janssen, MSD and Bayer HealthCare; and consulting fees and payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from MSD. M. Jevnikar reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen and MSD; and support for attending meetings from Janssen and MSD. T. Lacoste-Palasset reports support for attending meetings from Oxyvie. F. Picard reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Bayer, Boehringer Ingelheim/Lilly, Novartis, MSD, AstraZeneca and Novo Nordisk; and support for attending meetings from Bayer and Novartis. G. Prévôt reports consultancy fees from MSD; payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen; and support for attending meetings from GSK. S. Turquier reports consulting fees from Ferrer; payment or honoraria for lectures, presentations or educational events from MSD; and support for attending meetings from Ferrer, MSD and Janssen. M. Humbert reports grants from Gossamer and Merck; consulting fees from 35 Pharma, Aerovate, AOP Orphan, Chiesi, Ferrer, Gossamer, Janssen, Keros, Liquidia, Merck, Novartis, Respira, Roivant and United Therapeutics; payment or honoraria for lectures, presentations or educational events from Janssen and Merck; and participation on a data safety monitoring board or advisory board with 35 Pharma, Aerovate, Janssen, Keros, Merck, Novartis and United Therapeutics. O. Sitbon reports grants from Janssen, AOP Orphan, Aerovate, Ferrer, Gossamer Bio and Merck Sharp & Dohme; consulting fees from Janssen, Ferrer, AOP Health, Gossamer Bio, Merck Sharp & Dohme, Enzyvant, Liquidia, Respira Therapeutics and Roivant Sciences; payment or honoraria for lectures, presentations or educational events from Janssen, AOP Health, Ferrer, Merck Sharp & Dohme and Aerovate; support for attending meetings from Janssen, Gossamer Bio, Enzyvant and Ferrer (as a steering committee member); and participation on a data safety monitoring board or advisory board with Janssen, AOP Health, Ferrer, Merck Sharp & Dohme, Enzyvant, Gossamer Bio, Respira Therapeutics, Roivant Sciences and Liquidia. L. Savale reports payment or honoraria for lectures, presentations or educational events from MSD and Janssen and Janssen, and support for attending meetings from MSD and Janssen and Janssen. The remaining authors have no potential conflicts of interest to disclose.

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