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

Eur Respir J. 2019 Jan 24;53(1):1801887. doi: 10.1183/13993003.01887-2018. Print 2019 Jan.


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.

Publication types

  • Review

MeSH terms

  • Animals
  • Humans
  • Hypertension, Pulmonary / pathology*
  • Muscle, Smooth, Vascular / metabolism*
  • Muscle, Smooth, Vascular / pathology*
  • Pulmonary Artery / metabolism
  • Pulmonary Artery / pathology
  • Pulmonary Veins / metabolism
  • Pulmonary Veins / pathology
  • Signal Transduction / physiology
  • Vascular Remodeling*