Pulmonale Hypertonie assoziiert mit Lungenerkrankungen

Athiththan Yogeswaran; Dirk Skowasch; Vasile Foris; Finn Wilkens; Katarina Zeder; Claus Neurohr; Christian Opitz; Dennis Ladage; Elena Pfeuffer-Jovic; Max Wissmüller; Melanie Heberling; Stefan Krüger; Stephan Holt; Tobias J. Lange; Martin Schwaiblmair; H. Ardeschir Ghofrani; Jürgen Behr; Michael Halank

doi:10.1055/a-2625-4935

Pneumologie, Inhaltsverzeichnis

Pneumologie 2025; 79(10): 797-807
DOI: 10.1055/a-2625-4935

Übersicht

Pulmonale Hypertonie assoziiert mit Lungenerkrankungen

Pulmonary Hypertension Associated with Lung Diseases

Autoren

Athiththan Yogeswaran

¹Department of Internal Medicine, Universities of Gießen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Gießen (Germany), Institute for Lung Health (ILH), Cardio-Pulmonary Institute (CPI), Gießen, Deutschland
Dirk Skowasch

²Medizische Klinik und Poliklinik II, Universitätsklinikum Bonn, Bonn, Deutschland
Vasile Foris

³Division of Respiratory Medicine, Department of Internal Medicine, Lung Research Cluster, Medical University of Graz, Österreich

⁴Channing Division of Network Medicine, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
Finn Wilkens

⁵Thoraxklinik Heidelberg, Universitätsklinik Heidelberg Member of the German Center for Lung Research (DZL), Heidelberg, Deutschland
Katarina Zeder

³Division of Respiratory Medicine, Department of Internal Medicine, Lung Research Cluster, Medical University of Graz, Österreich

⁶University of Maryland, Institute of Health Computing, and School of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, Bethesda, MD, USA
Claus Neurohr

⁷Department of Pulmonology and Respiratory Medicine, Lung Center Stuttgart – Schillerhoehe Lung Clinic, affiliated to the Robert-Bosch-Hospital GmbH, Stuttgart, Deutschland; Comprehensive Pneumology Center, Member of the German Center for Lung Research (DZL), München, Deutschland
Christian Opitz

⁸Klinik für Innere Medizin – Kardiologie, DRK Kliniken Berlin Westend, Berlin, Deutschland
Dennis Ladage

⁹Klinik für Pneumologie, Kliniken Maria Hilf Mönchengladbach, Mönchengladbach, Deutschland
Elena Pfeuffer-Jovic

¹⁰Medizinische Klinik mit Schwerpunkt Pneumologie und Beatmungsmedizin, Klinikum Würzburg Mitte gGmbH, Würzburg, Deutschland
Max Wissmüller

¹¹Department of Cardiology, Heart Center at the University Hospital Cologne, and Cologne Cardiovascular Research Center (CCRC), Universität zu Köln, Köln, Deutschland
Melanie Heberling

¹²Division of Pneumology, Medical Department I, University Hospital Carl Gustav Carus of TU Dresden, Dresden, Deutschland
Stefan Krüger

¹³Klinik für Pneumologie, Kardiologie, internistische Intensivmedizin, Florence-Nightingale-Krankenhaus, Düsseldorf, Deutschland
Stephan Holt

¹⁴Praxis am Steintor, Recklinghausen, Recklinghausen
Tobias J. Lange

¹⁵Klinik für Innere Medizin II, Pneumologie und Beatmungsmedizin, Kreisklinik Bad Reichenhall; Fakultät für Medizin, Lehrstuhl für Innere Medizin II, Universität Regensburg, Regensburg, Deutschland
Martin Schwaiblmair

¹⁶I Medizinische Klinik, Sektion Pneumologie, Universitätsklinikum Augsburg, Augsburg, Deutschland
H. Ardeschir Ghofrani

¹Department of Internal Medicine, Universities of Gießen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Gießen (Germany), Institute for Lung Health (ILH), Cardio-Pulmonary Institute (CPI), Gießen, Deutschland
Jürgen Behr^*

¹⁷Department of Medicine V, LMU University Hospital, Comprehensive Pneumology Center, German Center for Lung Research, LMU, München, Deutschland
Michael Halank^*

¹²Division of Pneumology, Medical Department I, University Hospital Carl Gustav Carus of TU Dresden, Dresden, Deutschland

Abstract

Zusammenfassung

Die pulmonale Hypertonie bei chronischen Lungenerkrankungen (PH-CLD) ist eine der häufigsten, jedoch häufig unterdiagnostizierten Formen der pulmonalen Hypertonie (PH). Ihr Auftreten verschlechtert nachweislich Prognose und Lebensqualität der betroffenen Patient*innen. Neue epidemiologische Daten betonen die Heterogenität dieser Patientengruppe und zeigen, dass sowohl die zugrunde liegende Lungenerkrankung als auch der Schweregrad der PH für Prognose und Therapie entscheidend sind. Der 7. Weltkongress für pulmonale Hypertonie (WSPH) hat daher die Bedeutung einer systematischen Diagnostik und differenzierten Phänotypisierung von PH-CLD unterstrichen. Während einzelne Studien einen potenziellen Nutzen PH-spezifischer Therapien zeigen, berichten andere, etwa bei COPD, auch von negativen Effekten. Die vorliegende Stellungnahme der PH-DACH-Arbeitsgruppe fasst die wesentlichen Empfehlungen der 11. Task Force des 7. WSPH zusammen, bewertet die Evidenzlage kritisch und kommentiert gezielt Abweichungen vom Originaltext. Ziel ist es, einen Beitrag zur Entwicklung praxisnaher Diagnose- und Therapiealgorithmen für PH-CLD-Patienten zu leisten.

Abstract

Pulmonary hypertension associated with chronic lung diseases (PH-CLD) is one of the most common but frequently underdiagnosed forms of pulmonary hypertension (PH). Its presence has been consistently associated with worse prognosis and reduced quality of life for affected patients. Recent epidemiological data highlight the heterogeneity of this patient population, demonstrating that both the underlying lung disease and the severity of PH are critical determinants of prognosis and therapeutic decisions. The 7th World Symposium on Pulmonary Hypertension (WSPH) emphasized the importance of systematic diagnostic approaches and detailed phenotyping of PH-CLD. While some studies have shown potential benefits of PH-targeted therapies, others – particularly in COPD – have reported adverse outcomes. This position statement by the PH-DACH working group summarizes the key recommendations of the 11th Task Force of the 7th WSPH, critically assesses the current evidence, and comments specifically on deviations from the original text. The aim is to contribute to the development of practical diagnostic and therapeutic algorithms for patients with PH-CLD.

Schlüsselwörter

pulmonale Hypertonie - COPD - ILD - Lungenerkrankungen

Keywords

pulmonary hypertension - COPD - ILD - lung diseases

Volltext

Referenzen

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