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. 2025 Dec 1;11(6):00769-2025.
doi: 10.1183/23120541.00769-2025. eCollection 2025 Nov.

Long-term effects of medical treatment in patients with chronic thromboembolic pulmonary hypertension

Marion Delcroix  1 , Irene M Lang  2 , Andrea M D'Armini  3   4 , Elie Fadel  5 , Stefan Guth  6 , Stephen P Hoole  7 , David P Jenkins  7 , David G Kiely  8 , Nick H Kim  9 , Michael M Madani  10 , Hiromi Matsubara  11 , Kazuhiko Nakayama  12 , Aiko Ogawa  13 , Jaquelina S Ota-Arakaki  13 , Rozenn Quarck  1 , Roela Sadushi-Kolici  2 , Gérald Simonneau  14 , Christoph B Wiedenroth  6 , Bedrettin Yildizeli  15 , Eckhard Mayer  6 , Joanna Pepke-Zaba  7
Affiliations

Long-term effects of medical treatment in patients with chronic thromboembolic pulmonary hypertension

Marion Delcroix et al. ERJ Open Res. .

Abstract

Background: The New International Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Registry, conducted between 2015 and 2019, revealed the significant impact of pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty (BPA) on the long-term survival of CTEPH patients. The objective of the current pre-specified analysis was to evaluate the effect of medical treatment, including the approved oral guanylate cyclase stimulator (sGC) riociguat, in patients with or without mechanical intervention.

Methods: 1009 newly diagnosed patients were included in the study, with recruitment conducted at 34 centres across 20 countries. At the outset of the registry, sGC was available in 85% of the countries involved.

Results: 52% of all patients included were treated with pulmonary hypertension (PH) drugs. The proportion of patients receiving PH drugs was 38% among the 605 patients who underwent PEA, 78% among the 185 patients who underwent BPA and 76% among the 219 remaining patients who did not undergo PEA or BPA. In the "BPA" and "no PEA/BPA" groups, the 3-year survival was superior in patients who received sGC in comparison with other PH drugs. Cox regression confirmed that sGC treatment was associated with reduced mortality in the global cohort, in the BPA group and in the "no PEA/BPA" group.

Conclusion: This international CTEPH registry suggests that, although an increasing proportion of patients with CTEPH benefited from mechanical treatment, medical treatment with sGC may be associated with a survival advantage in patients undergoing BPA or no intervention. These observations confirm the results of previous randomised controlled trials in a real-world setting.

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Conflict of interest statement

Conflict of interest: M. Delcroix reports research grants from Janssen; speaker and consultant fees from Altavant, Acceleron, AOP, Bayer, Ferrer, Gossamer, INARI, Actelion-Janssen, J&J, United Therapeutics and MSD outside the submitted work, and all paid to her institution; and has been holder of a Janssen Chair for Pulmonary Hypertension at the KU Leuven. I.M. Lang has relationships with drug companies including AOP-Health, Actelion-Janssen, MSD, United Therapeutics, Bayer-Schering, Pulnovo, Medtronic, Neutrolis, Daiichi Sankyo, SciPharm Sàrl and Sanofi; in addition to being investigator in trials involving these companies, relationships include consultancy service, research grants and membership of scientific advisory boards. A.M. D'Armini reports consultancy fees from Actelion, Janssen, J&J, Acceleron, MDS, Merck and Bayer; and payment or honoraria for lectures, presentations, manuscript writing or educational events from Actelion, Janssen, J&J, Acceleron, MDS and Bayer. E. Fadel does not report any conflicts of interest. S. Guth reports consultancy fees and/or speaker fees, payment or honoraria for lectures, presentations, manuscript writing or educational events from Johnson & Johnson, Actelion/Janssen, Bayer AG, MSD and Pfizer; and support for attending meetings from Johnson & Johnson. S.P. Hoole does not report any conflicts of interest. D.P. Jenkins reports speaker fees and consultancy fees from Janssen, and is a board member for the ICA and on the scientific board for the World Symposium Pulmonary Hypertension. D.G. Kiely reports support for the present work from NIHR Sheffield Biomedical Research Centre; grants from Janssen Pharmaceuticals, National Institute of Health Research Sheffield Biomedical Research Centre and Ferrer; consultancy fees from Acceleron, Altavant, Ferrer, Janssen, Gossamer, Liquidia, Apollo, MSD and United Therapeutics; payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen Pharmaceuticals, Ferrer, Altavant, MSD and United Therapeutics; support for attending meetings from Janssen, Ferrer, MSD, United Therapeutics; participation on data safety monitoring boards or advisory boards with Janssen, MSD and Liquidia; and leadership roles with the Clinical Reference Group for Specialised Respiratory Medicine (NHS England) and UK National Audit of Pulmonary Hypertension. N.H. Kim reports having received speaker fees from Bayer, Janssen and Merck; consultancy fees from Bayer, Janssen, Merck, Gossamer Bio, Polarean, Pulnovo and United Therapeutics; and research support from Altavant and Gossamer Bio. M.M. Madani reports consultancy fees and royalties from Wexler Surgical; consultancy fees from Actelion-Janssen, Johnson & Johnson, Bayer and MSD and a leadership role with the ICA. H. Matsubara reports research grants from Nippon Shinyaku; speaker and consultant fees from Bayer, Janssen and MSD; speaker fees from Kaneka Medix, Mochida, Nippon Shinyaku, AOP Orphan and Nipro; payment for expert testimony from MSD; participation on a data safety monitoring board or advisory board for Bayer, Janssen, Mochida and MSD; and a leadership role with the ICA. K. Nakayama does not report any conflicts of interest. A. Ogawa does not report any conflicts of interest. J.S. Ota-Arakaki reports consulting fees, payment or honoraria for lectures, presentations, manuscript writing or educational events, support for attending meetings from Bayer, MSD and Janssen; participates on data safety monitoring boards or advisory boards with Bayer, MSD and Janssen; and holds a leadership role in the Brazilian Thoracic Society. R. Quark does not report any conflicts of interest. R. Sadushi-Kolici reports having received consultancy, speaker fees and support for attending meetings from AOP-Health, Actelion-Janssen and MSD. G. Simmoneau has received advisory board and speaker fees from Acceleron, Gossamer, Bayer, Janssen, MSD and Merck. C.B. Wiedenroth reports speaker fees and/or consultant honoraria from Johnson & Johnson, Actelion/Janssen, AOP Orphan Pharmaceuticals AG, Inari, Bayer AG, BTG, MSD, OrphaCare and Pfizer; and participation on a data safety monitoring board or advisory board for MSD. B. Yildizeli does not report any conflicts of interest. E. Mayer has received consultancy and/or speaker fees from Actelion/Janssen, Bayer and MSD. J. Pepke-Zaba reports research grants from MSD; consultancy fees from Ferrer, Gossamer, Janssen and MSD; payment or honoraria for lectures, presentations, manuscript writing or educational events from MSD; and support for attending meetings from AOP and Janssen.

Figures

FIGURE 1
FIGURE 1
Kaplan–Meier survival curve by intervention and with “no PEA/BPA” split by pulmonary hypertension (PH) drug regimens at any time. PEA: pulmonary endarterectomy; BPA: balloon pulmonary angioplasty; sGC: soluble guanylate cyclase stimulator. Comparisons: PEA versus BPA, p=0.074; PEA versus sGC, p=0.015; any of PEA, BPA or sGC versus any of other drugs or no drug, p<0.001; p >0.05 for all other comparisons.
FIGURE 2
FIGURE 2
Kaplan–Meier survival curves by procedure and by pulmonary hypertension (PH) drugs at any time. BPA: balloon pulmonary angioplasty; ERA: endothelin receptor antagonist; PCA: prostacyclin analogue; PDE5i: phosphodiesterase 5 inhibitor; PEA: pulmonary endarterectomy; sGC: soluble guanylate cyclase stimulator. Comparisons: patients undergoing BPA with versus without sGC treatment, p=0.040; patients undergoing BPA with versus without PCA treatment, p=0.006; p >0.05 for all other comparisons. “No” means no treatment with the drug class considered in the respective panel (sGC, PDE5i, ERA or PCA).
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