Pulmonary hypertension complicating portal hypertension

Am Rev Respir Dis. 1979 Oct;120(4):849-56. doi: 10.1164/arrd.1979.120.4.849.


We report 9 patients with pulmonary hypertension complicating portal hypertension. The cause of portal hypertension was cirrhosis in 7 patients, nodular regenerative hyperplasia of the liver in 1, and portal vein obstruction in 1. Six patients had been treated by portal-systemic shunting before the clinical onset of pulmonary hypertension. The interval between the first manifestation of portal hypertension and the recognition of pulmonary hypertension ranged from 2 to 15 years. Histologic examination in 1 of these patients revealed medial hypertrophy, concentric intimal proliferation, and plexiform lesions affecting the small pulmonary arteries. Pulmonary hypertension might result from the effect of a vasoconstrictive agent on the small pulmonary arteries or of a substance toxic to the walls of these vessels that is produced in the splanchnic territory, destroyed by the liver in normal subjects, and reaches the pulmonary arteries through portal-systemic shunts in these patients.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Cardiomegaly / diagnosis
  • Cardiomegaly / etiology
  • Electrocardiography
  • Female
  • Humans
  • Hypertension, Portal / complications*
  • Hypertension, Portal / therapy
  • Hypertension, Pulmonary / diagnostic imaging
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / pathology
  • Liver Cirrhosis / complications
  • Lung / pathology
  • Male
  • Middle Aged
  • Portacaval Shunt, Surgical / adverse effects
  • Portal Vein
  • Pulmonary Artery / diagnostic imaging
  • Radiography
  • Thrombosis / complications