[Definition and Classification of Pulmonary Hypertension].

Authors

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

Published in

Pneumologie (Stuttgart, Germany). Volume 79. Issue 10. Pages 723-731. Epub Oct 06, 2025.

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.

PMID:
41052526
Bibliographic data and abstract were imported from PubMed on 07 Oct 2025.

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