Definition und Klassifikation der pulmonalen Hypertonie

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

Publication History

Article published online:
06 October 2025

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