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

M Humbert, C Guignabert, S Bonnet… - European …, 2019 - Eur Respiratory Soc
M Humbert, C Guignabert, S Bonnet, P Dorfmüller, JR Klinger, MR Nicolls, AJ Olschewski
European Respiratory Journal, 2019Eur Respiratory Soc
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 (eg 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 …
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.
European Respiratory Society