Pathology and Pathobiology of Pulmonary Hypertension: State of the Art and Research Perspectives

Overview

Journal Eur Respir J

Specialty Pulmonary Medicine

Date 2018 Dec 15

PMID 30545970

Citations 522

Authors

Marc Humbert

Christophe Guignabert

Sebastien Bonnet

Peter Dorfmuller

James R Klinger

Mark R Nicolls

Andrea J Olschewski

Soni S Pullamsetti

Ralph T Schermuly

Kurt R Stenmark

Marlene Rabinovitch

Affiliations

Soon will be listed here.

Abstract

Clinical and translational research has played a major role in advancing our understanding of pulmonary hypertension (PH), including pulmonary arterial hypertension and other forms of PH with severe vascular remodelling ( chronic thromboembolic PH and pulmonary veno-occlusive disease). However, PH remains an incurable condition with a high mortality rate, underscoring the need for a better transfer of novel scientific knowledge into healthcare interventions. Herein, we review recent findings in pathology (with the questioning of the strict morphological categorisation of various forms of PH into pre- or post-capillary involvement of pulmonary vessels) and cellular mechanisms contributing to the onset and progression of pulmonary vascular remodelling associated with various forms of PH. We also discuss ways to improve management and to support and optimise drug development in this research field.

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