Pathology and pathobiology of pulmonary hypertension

Semin Respir Crit Care Med. 2013 Oct;34(5):551-9. doi: 10.1055/s-0033-1356496. Epub 2013 Sep 13.


Pulmonary hypertension is a devastating, life-threatening disorder with no curative options, characterized by elevated pulmonary vascular resistance and secondary right ventricular failure. Although the etiologies of pulmonary arterial hypertension are multiple and its pathogenesis is complex, there is growing evidence that inflammation, endothelial dysfunction, aberrant vascular wall cell proliferation, as well as mutations in the bone morphogenetic protein receptor type 2 gene play a crucial role in triggering pathological vascular remodeling. The present article outlines the current understanding of this disease from the point of view of pathology and pathobiology.

Publication types

  • Review

MeSH terms

  • Endothelial Cells
  • Endothelium, Vascular / immunology
  • Endothelium, Vascular / pathology*
  • Endothelium, Vascular / physiopathology
  • Familial Primary Pulmonary Hypertension
  • Fibrosis
  • Genetic Predisposition to Disease
  • Humans
  • Hypertension, Pulmonary / genetics
  • Hypertension, Pulmonary / pathology*
  • Hypertension, Pulmonary / physiopathology
  • Inflammation / physiopathology
  • Lung / blood supply
  • Lung / immunology
  • Lung / pathology*
  • Muscle, Smooth, Vascular / pathology*
  • Pulmonary Artery / immunology
  • Pulmonary Artery / pathology*
  • Pulmonary Artery / physiopathology
  • Pulmonary Circulation / immunology