Definition und Klassifikation der pulmonalen Hypertonie

Philipp Douschan; Ann-Sophie Kaemmerer-Suleiman; Benjamin Egenlauf; Hans-Jürgen Seyfarth; Katarina Zeder; Laura Mayer; Max Wissmüller; Melanie Heberling; Michael Huntgeburth; Michaela Barnikel; Panagiota Xanthouli; Stephan Sorichter; Teresa John; Silvia Ulrich; Gabor Kovacs

doi:10.1055/a-2625-4769

Pneumologie, Table of Contents

Pneumologie 2025; 79(10): 723-731
DOI: 10.1055/a-2625-4769

Übersicht

Definition und Klassifikation der pulmonalen Hypertonie

Definition and Classification of Pulmonary Hypertension

Authors

Philipp Douschan

¹Abteilung für Pulmonologie, Lung Research Cluster, Medizinische Universität Graz, Graz, Österreich
Ann-Sophie Kaemmerer-Suleiman

²Herzchirurgische Klinik, Universitätsklinikum Erlangen, Erlangen, Deutschland
Benjamin Egenlauf

³Zentrum für pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg, Heidelberg, Deutschland
Hans-Jürgen Seyfarth

⁴Abteilung Pneumologie, Medizinische Klinik und Poliklinik für Onkologie, Gastroenterologie, Hepatologie und Pneumologie der Universität Leipzig, Leipzig, Deutschland
Katarina Zeder

¹Abteilung für Pulmonologie, Lung Research Cluster, Medizinische Universität Graz, Graz, Österreich

⁵University of Maryland, Institute for Health Computing, Bethesda, MD, USA
Laura Mayer

⁶Klinik für Pneumologie, Universitätsspital Zürich, Zürich, Schweiz
Max Wissmüller

⁷Klinik III für Innere Medizin – Allgemeine und interventionelle Kardiologie, Elektrophysiologie, Angiologie, Pneumologie und internistische Intensivmedizin, Uniklinik Köln, Köln, Deutschland
Melanie Heberling

⁸Medizinische Klinik I – Pneumologie, Universitätsklinikum Dresden, Dresden, Deutschland
Michael Huntgeburth

⁹Internationales Zentrum für Erwachsene mit angeborenen Herzfehlern (EMAH), München, Deutschland
Michaela Barnikel

¹⁰LMU Klinikum München, Medizinische Klinik und Poliklinik V, München, Deutschland. Comprehensive Pneumology Center (CPC-M), Mitglied des Deutschen Zentrums für Lungenforschung (DZL)
Panagiota Xanthouli

³Zentrum für pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg, Heidelberg, Deutschland

¹¹Medizinische Klinik V, Rheumaambulanz, Universitätsklinikum Heidelberg, Heidelberg, Deutschland
Stephan Sorichter

¹²Klinik für Pneumologie, Infektiologie und Beatmungsmedizin, St. Josefskrankenhaus, Freiburg, Deutschland
Teresa John

¹Abteilung für Pulmonologie, Lung Research Cluster, Medizinische Universität Graz, Graz, Österreich
Silvia Ulrich

⁶Klinik für Pneumologie, Universitätsspital Zürich, Zürich, Schweiz
Gabor Kovacs

¹Abteilung für Pulmonologie, Lung Research Cluster, Medizinische Universität Graz, Graz, Österreich

Abstract

Zusammenfassung

Die pulmonale Hypertonie (PH) stellt einen hämodynamisch definierten pathologischen Zustand dar, welcher durch einen mittleren pulmonalarteriellen Druck (mPAP) > 20 mmHg charakterisiert ist. Die differenzialdiagnostische Zuordnung erfolgt anhand des pulmonalarteriellen Wedge-Drucks (PAWP) und des pulmonalen Gefäßwiderstands (PVR): präkapilläre PH: PAWP ≤ 15 mmHg bei gleichzeitig erhöhtem PVR > 2 Wood Units (WU), isolierte postkapilläre PH: PAWP > 15 mmHg, PVR ≤ 2 WU und kombinierte post- und präkapilläre PH: PAWP > 15 mmHg und PVR > 2 WU.

Die belastungsinduzierte PH ist definiert als normwertiger mPAP in Ruhe, jedoch pathologischer Drucksteigerung unter körperlicher Belastung, definiert durch eine Steigung des mPAP relativ zum Herzzeitvolumen (HZV) von > 3 mmHg/L/min.

Die etablierte klinische Klassifikation der PH bleibt in ihrer Grundstruktur mit 5 Hauptgruppen erhalten. Modifikationen beinhalten: Re-Etablierung der Subgruppe der Langzeitresponder auf Kalziumkanalblocker innerhalb der idiopathischen pulmonalarteriellen Hypertonie (IPAH), Erweiterung der Gruppe 2 durch zusätzliche Subkategorien bei PH infolge Linksherzerkrankungen und verfeinerte Untergliederung der Gruppe 3 auf Basis spezifischer pulmonaler Grunderkrankungen statt rein funktioneller Einschränkungen. Die Wirkstoffe Mitomycin-C und Carfilzomib wurden neu in die Liste der Pharmaka mit gesicherter Assoziation zur Entwicklung einer PAH aufgenommen.

Abstract

Pulmonary Hypertension (PH) is characterized as a hemodynamic disorder defined by a mean pulmonary arterial pressure (mPAP) exceeding 20 mmHg at rest. Classification into distinct subtypes is guided by measurements of pulmonary arterial wedge pressure (PAWP) and pulmonary vascular resistance (PVR): Precapillary PH: PAWP ≤ 15 mmHg accompanied by PVR > 2 Wood Units (WU), isolated Postcapillary PH: PAWP > 15 mmHg with PVR ≤ 2 WU and combined Pre- and Postcapillary PH: PAWP > 15 mmHg with PVR > 2 WU.

Exercise-Induced PH refers to a pathophysiological condition in which resting mPAP is within normal limits but exhibits an exaggerated rise during physical exertion. This is quantified by a slope of mPAP relative to cardiac output exceeding 3 mmHg per liter per minute between rest and activity.

The foundational framework for the clinical classification of PH, comprising five principal groups, remains unchanged. Nevertheless, several updates have been introduced: Reintegration of long-term responders to calcium channel blockers as a distinct subset within idiopathic pulmonary arterial hypertension (IPAH), Inclusion of new subcategories under Group 2 PH, which encompasses PH associated with left heart disease and Revision of Group 3 PH to categorize patients based on underlying pulmonary pathology rather than solely functional impairment.

Additionally, Mitomycin-C and Carfilzomib have been recognized as pharmacologic agents with a confirmed causal relationship to the development of pulmonary arterial hypertension (PAH) and have thus been added to the list of definitively associated drugs.

Schlüsselwörter

Hämodynamik - Belastungs-PH - PH-Untergruppen

Keywords

hemodynamics - exercise PH - PH subgroups

Full Text

References

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