Risikostratifizierung und Behandlungsziele bei pulmonalarterieller Hypertonie

Horst Olschewski; Michaela Barnikel; David Pittrow; Martin Schwaiblmair; Mathieu Suleiman; Athiththan Yogeswaran; Panagiota Xanthouli; Pauline Gieseke; Philipp Douschan; Ann-Sophie Kaemmerer-Suleiman; Stefan Krüger; Stephan Sorichter; Teresa John; Silvia Ulrich

doi:10.1055/a-2625-4844

Pneumologie, Inhaltsverzeichnis

Pneumologie 2025; 79(10): 751-761
DOI: 10.1055/a-2625-4844

Übersicht

Risikostratifizierung und Behandlungsziele bei pulmonalarterieller Hypertonie

Risk Stratification and Treatment Goals for Pulmonary Arterial Hypertension

Authors

Horst Olschewski

¹Sigmund Freud Private University, Wien, Österreich und Charité University Medicine, Berlin, Deutschland
Michaela Barnikel

²LMU Klinikum München, Medizinische Klinik und Poliklinik V, München, Deutschland. Comprehensive Pneumology Center (CPC-M), Mitglied des Deutschen Zentrums für Lungenforschung (DZL), Deutschland
David Pittrow

³Institut für Klinische Pharmakologie, Medizinische Fakultät, Technische Universität, Dresden, Deutschland
Martin Schwaiblmair

⁴Medizinische Klinik, Sektion Pneumologie, Universitätsklinikum Augsburg, Augsburg, Deutschland
Mathieu Suleiman

⁵Klinik für Herzchirurgie, Universitätsklinikum Erlangen, Erlangen, Deutschland
Athiththan Yogeswaran

⁶Department of Internal Medicine, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Gießen, Deutschland; Institute for Lung Health (ILH), Cardio-Pulmonary Institute (CPI), Gießen, Deutschland
Panagiota Xanthouli

⁷Zentrum für Pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg, Heidelberg, Deutschland

⁸Medizinische Klinik V, Rheumaambulanz, Universitätsklinikum Heidelberg, Heidelberg, Deutschland
Pauline Gieseke

⁷Zentrum für Pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg, Heidelberg, Deutschland
Philipp Douschan

⁹Universitätsklinik Innere Medizin, Klinische Abteilung für Pneumologie, Medizinische Universität Graz, Österreich
Ann-Sophie Kaemmerer-Suleiman

⁵Klinik für Herzchirurgie, Universitätsklinikum Erlangen, Erlangen, Deutschland
Stefan Krüger

¹⁰Klinik für Pneumologie, Kardiologie und Internistische Intensivmedizin, Florence-Nightingale-Krankenhaus, Düsseldorf und Klinik für Kardiologie, Pneumologie, Angiologie, Universitätsklinikum, Düsseldorf, Deutschland
Stephan Sorichter

¹¹Klinik für Pneumologie, Infektiologie und Beatmungsmedizin, St. Josefskrankenhaus, Freiburg, Deutschland
Teresa John

⁹Universitätsklinik Innere Medizin, Klinische Abteilung für Pneumologie, Medizinische Universität Graz, Österreich
Silvia Ulrich

¹²Klinik für Pneumologie, Universität und Universitätsspital Zürich, Schweiz

Abstract

Zusammenfassung

Die Risikostratifizierung hat eine wichtige Rolle bei der Vorhersage von Ergebnissen und der Steuerung der Behandlung von Patient*innen mit pulmonalarterieller Hypertonie (PAH). Die 3 wichtigsten nicht-invasiven prädiktiven Prognosefaktoren sind die Funktionsklasse der Weltgesundheitsorganisation, die 6-Minuten-Gehstrecke und die natriuretischen Peptide. Alle 3 sind in allen derzeit validierten Risikostratifizierungsinstrumenten enthalten. Aufgrund von Einschränkungen, die hauptsächlich mit der reduzierten Spezifität der Schwere der PAH zusammenhängen, sind diese Variablen jedoch nicht immer ausreichend, um individuelle Behandlungsentscheidungen zu treffen. Darüber hinaus wird erwartet, dass Marker, die mit pulmonal-vaskulärem Remodelling assoziiert sind, mit effektiven Kombinations- und neuen PAH-Therapien zunehmend relevant werden, um die Behandlung von Patient*innen mit PAH zu steuern. Während das Erreichen eines niedrigen Mortalitätsrisikos, bewertet mit einem validierten Risikoinstrument, ein wichtiges Behandlungsziel bleibt, deuten vorläufige Daten darauf hin, dass invasive Hämodynamik und kardiale Bildgebung einen zusätzlichen Wert bei der Steuerung von Behandlungsentscheidungen bieten können.

Abstract

Risk stratification plays an important role in predicting outcomes and guiding treatment decisions of patients with pulmonary arterial hypertension (PAH). The three most important non-invasive predictive prognostic factors are the World Health Organization functional class, the 6-minute walk distance, and natriuretic peptides. These three are included in all currently validated risk stratification tools. However, due to limitations primarily related to the reduced specificity of PAH severity, these variables are not always sufficient to guide individual treatment decisions. Furthermore, with effective combination and new PAH therapies, markers associated with pulmonary vascular remodeling are expected to become increasingly relevant for guiding the treatment of patients with PAH. While achieving a low mortality risk, measured with a validated risk assessment tool, remains an important treatment goal, preliminary data suggest that invasive hemodynamics and cardiac imaging may provide additional value in guiding treatment decisions.

Schlüsselwörter

pulmonale Hypertonie - pulmonalarterielle Hypertonie - Risikostratifizierung - Prognose - Prognosefaktoren

Keywords

pulmonary hypertension - pulmonary arterial hypertension - risk stratification - prognosis - outcome predictors

Volltext

Referenzen

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