Risk stratification and response to therapy in patients with pulmonary arterial hypertension and comorbidities: A COMPERA analysis.


Journal

The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
ISSN: 1557-3117
Titre abrégé: J Heart Lung Transplant
Pays: United States
ID NLM: 9102703

Informations de publication

Date de publication:
01 2023
Historique:
received: 28 06 2022
revised: 20 09 2022
accepted: 04 10 2022
pubmed: 5 11 2022
medline: 24 12 2022
entrez: 4 11 2022
Statut: ppublish

Résumé

A diagnosis of idiopathic pulmonary arterial hypertension (IPAH) is frequently made in elderly patients who present with comorbidities, especially hypertension, coronary heart disease, diabetes mellitus, and obesity. It is unknown to what extent the presence of these comorbidities affects the response to PAH therapies and whether risk stratification predicts outcome in patients with comorbidities. We assessed the database of COMPERA, a European pulmonary hypertension registry, to determine changes after initiation of PAH therapy in WHO functional class (FC), 6-minute walking distance (6MWD), brain natriuretic peptide (BNP) or N-terminal fragment of probrain natriuretic peptide (NT-pro-BNP), and mortality risk assessed by a 4-strata model in patients with IPAH and no comorbidities, 1-2 comorbidities and 3-4 comorbidities. The analysis was based on 1,120 IPAH patients (n = 208 [19%] without comorbidities, n = 641 [57%] with 1-2 comorbidities, and n = 271 [24%] with 3-4 comorbidities). Improvements in FC, 6MWD, BNP/NT-pro-BNP, and mortality risk from baseline to first follow-up were significantly larger in patients with no comorbidities than in patients with comorbidities, while they were not significantly different in patients with 1-2 and 3-4 comorbidities. The 4-strata risk tool predicted survival in patients without comorbidities as well as in patients with 1-2 or 3-4 comorbidities. Our data suggest that patients with IPAH and comorbidities benefit from PAH medication with improvements in FC, 6MWD, BNP/NT-pro-BNP, and mortality risk, albeit to a lesser extent than patients without comorbidities. The 4-strata risk tool predicted outcome in patients with IPAH irrespective of the presence of comorbidities.

Sections du résumé

BACKGROUND
A diagnosis of idiopathic pulmonary arterial hypertension (IPAH) is frequently made in elderly patients who present with comorbidities, especially hypertension, coronary heart disease, diabetes mellitus, and obesity. It is unknown to what extent the presence of these comorbidities affects the response to PAH therapies and whether risk stratification predicts outcome in patients with comorbidities.
METHODS
We assessed the database of COMPERA, a European pulmonary hypertension registry, to determine changes after initiation of PAH therapy in WHO functional class (FC), 6-minute walking distance (6MWD), brain natriuretic peptide (BNP) or N-terminal fragment of probrain natriuretic peptide (NT-pro-BNP), and mortality risk assessed by a 4-strata model in patients with IPAH and no comorbidities, 1-2 comorbidities and 3-4 comorbidities.
RESULTS
The analysis was based on 1,120 IPAH patients (n = 208 [19%] without comorbidities, n = 641 [57%] with 1-2 comorbidities, and n = 271 [24%] with 3-4 comorbidities). Improvements in FC, 6MWD, BNP/NT-pro-BNP, and mortality risk from baseline to first follow-up were significantly larger in patients with no comorbidities than in patients with comorbidities, while they were not significantly different in patients with 1-2 and 3-4 comorbidities. The 4-strata risk tool predicted survival in patients without comorbidities as well as in patients with 1-2 or 3-4 comorbidities.
CONCLUSIONS
Our data suggest that patients with IPAH and comorbidities benefit from PAH medication with improvements in FC, 6MWD, BNP/NT-pro-BNP, and mortality risk, albeit to a lesser extent than patients without comorbidities. The 4-strata risk tool predicted outcome in patients with IPAH irrespective of the presence of comorbidities.

Identifiants

pubmed: 36333206
pii: S1053-2498(22)02169-6
doi: 10.1016/j.healun.2022.10.003
pii:
doi:

Substances chimiques

Natriuretic Peptide, Brain 114471-18-0
Peptide Fragments 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

102-114

Informations de copyright

Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.

Auteurs

Stephan Rosenkranz (S)

Clinic III for Internal Medicine (Cardiology) and Center for Molecular Medicine (CMMC), and the Cologne Cardiovascular Research Center (CCRC), University of Cologne, Cologne, Germany.

Christine Pausch (C)

GWT-TUD GmbH, Epidemiological Centre, Dresden, Germany.

John G Coghlan (JG)

Department of Cardiology, Royal Free Hospital, London, United Kingdom.

Doerte Huscher (D)

Institute of Biometry and Clinical Epidemiology, and Berlin Institute of Health, Charité-Universitätsmedizin, Berlin, Germany.

David Pittrow (D)

GWT-TUD GmbH, Epidemiological Centre, Dresden, Germany; Institute for Clinical Pharmacology, Medical Faculty, Technical University, Dresden, Germany.

Ekkehard Grünig (E)

Center for Pulmonary Hypertension, Thoraxklinik at Heidelberg University Hospital, Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), Heidelberg, Germany.

Gerd Staehler (G)

Fachklinik Löwenstein, Löwenstein, Germany.

Carmine Dario Vizza (CD)

Dipartimento di Scienze Cliniche Internistiche, Anestiologiche e Cardiolohiche, Sapienza, University of Rome; Rome, Italy.

Henning Gall (H)

Department of Internal Medicine, Justus-Liebig-University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Giessen, Germany; German Center of Lung Research (DZL), Germany.

Oliver Distler (O)

Department of Rheumatology, University Hospital, Zurich, Switzerland.

Marion Delcroix (M)

Clinical Department of Respiratory Diseases, University Hospitals of Leuven and Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), Department of Chronic Diseases and Metabolism (CHROMETA), KU Leuven - University of Leuven, Leuven, Belgium.

Hossain A Ghofrani (HA)

Department of Internal Medicine, Justus-Liebig-University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Giessen, Germany; German Center of Lung Research (DZL), Germany; Department of Medicine, Imperial College London, London, United Kingdom.

Ralf Ewert (R)

Clinic of Internal Medicine, Department of Respiratory Medicine, Universitätsmedizin Greifswald, Germany.

Hans-Joachim Kabitz (HJ)

Gemeinnützige Krankenhausbetriebsgesellschaft Konstanz mbH, Medizinische Klinik II, Konstanz, Germany.

Dirk Skowasch (D)

Universitätsklinikum Bonn, Medizinische Klinik und Poliklinik II, Innere Medizin - Kardiologie/Pneumologie, Bonn.

Juergen Behr (J)

Comprehensive Pneumology Center, Lungenforschungsambulanz, Helmholtz Zentrum, München, Germany; Department of Medicine V, University Hospital, LMU Munich, Comprehensive Pneumology Center Munich (CPC-M), Munich, Germany.

Katrin Milger (K)

Department of Medicine V, University Hospital, LMU Munich, Comprehensive Pneumology Center Munich (CPC-M), Munich, Germany.

Michael Halank (M)

Universitätsklinikum Carl Gustav Carus der Technischen Universität Dresden, Medizinische Klinik und Poliklinik I, Dresden, Germany.

Heinrike Wilkens (H)

Klinik für Innere Medizin V, Pneumologie, Universitätsklinikum des Saarlandes, Homburg, Germany.

Hans-Jürgen Seyfarth (HJ)

Universitätsklinikum Leipzig, Medizinische Klinik und Poliklinik II, Abteilung für Pneumologie, Leipzig, Germany.

Matthias Held (M)

Department of Internal Medicine, Respiratory Medicine and Ventilatory Support, Medical Mission Hospital, Central Clinic Würzburg, Würzburg, Germany.

Laura Scelsi (L)

Fondazione IRCSS S. Matteo Pavia, Division of Cardiology Stolfo Davide, Azienda Sanitaria Universitaria Giuliano Isontina, Pavia, Italy.

Claus Neurohr (C)

Department of Pulmonology and Respiratory Medicine, Robert-Bosch-Krankenhaus Stuttgart, Stuttgart, Germany.

Anton Vonk-Noordegraaf (A)

Amsterdam UMC, Vrije Universiteit Amsterdam, dept of Pulmonary Medicine, Amsterdam Cardiovascular Sciences, Amsterdam, Netherlands.

Silvia Ulrich (S)

Clinic of Pulmonology, University Hospital of Zurich, Zurich, Switzerland.

Hans Klose (H)

Department of Respiratory Medicine, Eppendorf University Hospital, Hamburg, Germany.

Martin Claussen (M)

LungenClinic Grosshansdorf, Fachabteilung Pneumologie, Großhansdorf, Germany.

Stephan Eisenmann (S)

Department of Respiratory Medicine, Universitätsklinikum Halle, Halle, Germany.

Kai-Helge Schmidt (KH)

Department of Cardiology and Center of Thrombosis and Hemostasis (CTH); University Medical Center Mainz, Germany.

Bjoern Andrew Remppis (BA)

Herz- und Gefäßzentrum Bad Bevensen, Bad Bevensen, Germany.

Andris Skride (A)

Riga Stradiņš University, Rare Diseases Unit VSIA Pauls Stradins Clinical University Hospital, Riga, Latvia.

Elena Jureviciene (E)

Faculty of Medicine of Vilnius University, Competence Centre of Pulmonary Hypertension, Vilnius University Hospital Santaros klinikos, Vilnius, Lithuania.

Lina Gumbiene (L)

Faculty of Medicine of Vilnius University, Competence Centre of Pulmonary Hypertension, Vilnius University Hospital Santaros klinikos, Vilnius, Lithuania.

Skaidrius Miliauskas (S)

Department of Pulmonology, Lithuanian University of Health Sciences, Kaunas, Lithuania.

Judith Löffler-Ragg (J)

Department of Internal Medicine II, Medical University of Innsbruck, Innsbruck, Austria.

Tobias J Lange (TJ)

Department of Internal Medicine II, University Medical Center Regensburg, Regensburg, Germany.

Karen M Olsson (KM)

German Center of Lung Research (DZL), Germany; Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany.

Marius M Hoeper (MM)

German Center of Lung Research (DZL), Germany; Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany. Electronic address: hoeper.marius@mh-hannover.de.

Christian Opitz (C)

Department of Cardiology, DRK Kliniken Berlin Westend, Berlin, Germany.

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