Pathologic assessment of vasculopathies in pulmonary hypertension

J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):25S-32S. doi: 10.1016/j.jacc.2004.02.033.


Pulmonary arterial hypertension (PAH) includes various forms of pulmonary hypertension of different etiology but similar clinical presentation and functional derangement. Histopathological vascular changes in all forms of PAH are qualitatively similar but with quantitative differences in the distribution and prevalence of pathological changes in various portions of the pulmonary vascular bed. The documentation of these topographic variations in the response of the pulmonary vasculature to injury may be important to understand the pathogenesis of the various subsets of PAH. To standardize the precise histopathological documentation of the pulmonary vasculopathy in PAH we propose a histopathological classification that includes both the predominant segment of the pulmonary vasculature affected and the possible coexistence of pathological changes in other vascular segments.

Publication types

  • Review

MeSH terms

  • Humans
  • Hypertension, Pulmonary / pathology*
  • Hypertension, Pulmonary / physiopathology
  • Lung / blood supply*
  • Muscle, Smooth, Vascular / pathology
  • Muscle, Smooth, Vascular / physiopathology
  • Pulmonary Artery / pathology
  • Pulmonary Artery / physiopathology
  • Pulmonary Circulation / physiology
  • Tunica Intima / pathology
  • Tunica Intima / physiopathology
  • Vascular Diseases / pathology*
  • Vascular Resistance / physiology
  • Vasoconstriction / physiology