Primary pulmonary hypertension and cirrhosis: are they related?

Am Rev Respir Dis. 1983 Apr;127(4):437-41. doi: 10.1164/arrd.1983.127.4.437.


The relationship of primary pulmonary hypertension to longstanding portal hypertension secondary to hepatic cirrhosis was examined. An unselected series of 17,901 autopsied patients older than 1 yr of age showed that primary pulmonary hypertension occurred with a prevalence of 0.13% in all patients, but with a prevalence of 0.73% among patients with cirrhosis (p less than 0.001). A clinical series of 2,459 patients with biopsy-proved hepatic cirrhosis showed a prevalence of primary pulmonary hypertension of 0.61%, also significant (p less than 0.001) when compared with the prevalence among all patients older than 1 yr of age at autopsy. These data appear to exclude a coincidental association of cirrhosis with the development of the vascular lesions of primary pulmonary hypertension.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Autopsy
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Hypertension, Portal / complications
  • Hypertension, Pulmonary / epidemiology
  • Hypertension, Pulmonary / etiology*
  • Infant
  • Liver Cirrhosis / complications*
  • Liver Cirrhosis / epidemiology
  • Male
  • Middle Aged