Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives

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 ( e.g. 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.

Keywords

Affiliated Institutions

• Rhode Island Hospital US
• Max Planck Institute for Heart and Lung Research DE
• Assistance Publique – Hôpitaux de Paris FR
• Inserm FR
• Hôpital Marie Lannelongue FR
• Bicêtre Hospital FR
• Stanford University US
• Justus-Liebig-Universität Gießen DE
• Université Paris-Sud FR
• Université Laval CA
• Cardiovascular Institute of the South US
• University of Colorado Denver US
• Brown University US
• Cardio-Pulmonary Institute DE
• Universities of Giessen and Marburg Lung Center DE
• Université Paris-Saclay FR
• Ludwig Boltzmann Institute for Lung Vascular Research AT
• Medical University of Graz AT
• AR2i FR
• Institut Universitaire de Cardiologie et de Pneumologie de Québec CA
• German Center for Lung Research DE

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