Molecular pathogenesis of pulmonary arterial hypertension

J Clin Invest. 2008 Jul;118(7):2372-9. doi: 10.1172/JCI33452.


Recent investigations have suggested that it might be possible to reverse the pathology of pulmonary arterial hypertension (PAH), a disorder that can be rapidly progressive and fatal despite current treatments including i.v. prostacyclin. This review will address the cellular and molecular processes implicated in clinical, genetic, and experimental studies as underlying the pulmonary vascular abnormalities associated with PAH. Emerging treatments are aimed at inducing apoptosis of abnormal vascular cells that obstruct blood flow and at promoting regeneration of "lost" distal vasculature.

Publication types

  • Review

MeSH terms

  • Animals
  • Bone Morphogenetic Protein Receptors, Type II / physiology
  • Humans
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / physiopathology*
  • Hypertension, Pulmonary / therapy
  • Inflammation Mediators / physiology
  • Ion Channels / physiology
  • Leukocyte Elastase / metabolism
  • Mesenchymal Stem Cells / physiology
  • Models, Biological*
  • Vasodilator Agents / therapeutic use


  • Inflammation Mediators
  • Ion Channels
  • Vasodilator Agents
  • Bone Morphogenetic Protein Receptors, Type II
  • Leukocyte Elastase