Risk Assessment in Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension
- Marc Humbert1,2,3⇑,
- Harrison W. Farber4,
- Hossein-Ardeschir Ghofrani5,6,
- Raymond L. Benza7,
- Dennis Busse8,
- Christian Meier9 and
- Marius M. Hoeper10
- 1University Paris-Sud, Faculté de Médecine, Université Paris Saclay, Le Kremlin-Bicêtre, France
- 2Assistance-Publique–Hôpitaux de Paris, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
- 3INSERM Unité 999, Le Kremlin–Bicêtre, France
- 4Division of Pulmonary, Critical Care and Sleep Medicine, Tufts Medical Center, Boston, Massachusetts, United States of America
- 5University of Giessen and Marburg Lung Center, Giessen, Germany, member of the German Center for Lung Research (DZL)
- 6Department of Medicine, Imperial College London, London, United Kingdom
- 7Cardiovascular Institute, Allegheny General Hospital, Pittsburgh, Pennsylvania
- 8Chrestos Concept GmbH & Co. KG, Essen, Germany
- 9Bayer AG, Berlin, Germany
- 10Clinic for Respiratory Medicine, Hannover Medical School, Hannover, Germany, member of the German Center for Lung Research (DZL)
- Prof. Marc Humbert, MD, PhD Service de Pneumologie, Hôpital Bicêtre, 78, Rue du général Leclerc, 94270 Le Kremlin-Bicêtre, France. E-mail: marc.humbert{at}aphp.fr
Abstract
Rationale Current pulmonary hypertension treatment guidelines recommend use of a risk stratification model encompassing a range of parameters, allowing patients to be categorised as low, intermediate, or high risk. Three abbreviated versions of this risk stratification model were previously evaluated in patients with pulmonary arterial hypertension in the French, Swedish, and COMPERA registries.
Objective To investigate 3 abbreviated risk stratification methods for patients with mostly prevalent pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension, in patients from the PATENT-1/-2 and CHEST-1/-2 studies of riociguat.
Methods Risk was assessed at baseline and at follow-up in PATENT-1 and CHEST-1. Survival and clinical worsening-free survival were assessed in patients in each risk group/strata.
Measurements and Main Results With all 3 methods, riociguat improved risk group/strata in patients with pulmonary arterial hypertension after 12 weeks. The French non-invasive and Swedish/COMPERA methods discriminated prognosis for survival and clinical worsening-free survival at both baseline and follow-up. Furthermore, patients achieving ≥1 low-risk criteria or a low-risk stratum at follow-up had a significantly reduced risk of death and clinical worsening, compared with patients achieving no low-risk criteria or an intermediate-risk stratum. Similar results were obtained in patients with inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension.
Conclusions This analysis confirms and extends the results of the registry analyses, supporting the value of goal-oriented treatment in pulmonary arterial hypertension. Further assessment of these methods in patients with chronic thromboembolic pulmonary hypertension is warranted.
Footnotes
This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.
Conflict of interest: Dr. FARBER reports grants from Actelion, Gilead, United Therapeutics, personal fees from Actelion, Bayer, Bellerophon, Gilead, United Therapeutics, during the conduct of the study.
Conflict of interest: Dr. GHOFRANI reports grants and personal fees from Actelion, grants and personal fees from Bayer AG, grants and personal fees from Ergonex, grants and personal fees from Pfizer, personal fees from Gilead, personal fees from GSK, personal fees from Merck, personal fees from Novartis, outside the submitted work.
Conflict of interest: Dr. BENZA reports grants from Belleraphon, grants from Bayer AG, grants from Actelion, grants from EIGER, during the conduct of the study; .Dr. BENZA reports grants from Belleraphon, grants from Bayer AG, grants from Actelion, grants from EIGER, during the conduct of the study.
Conflict of interest: Dr. BUSSE reports other from Bayer AG, during the conduct of the study.
Conflict of interest: Dr. MEIER reports other from Bayer AG, during the conduct of the study.
Conflict of interest: Dr. HOEPER reports consultancy fees from Actelion, Bayer AG, GSK, and Pfizer, during the conduct of the study.
Conflict of interest: Dr. HUMBERT reports personal fees from Bayer, personal fees from Merck, during the conduct of the study; personal fees from Actelion, and support from GSK, from Johnson & Johnson, from United Therapeutics, outside the submitted work.
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- Copyright ©ERS 2019