Portopulmonary hypertension

Portopulmonary hypertension (POPH/PPHTN) refers to pulmonary artery hypertension that develops in the setting of portal hypertension (with or without underlying liver disease). It falls under group 1.4 under the Dana point 2008 pulmonary hypertension classification system.

The prevalence of portopulmonary hypertension ranges from 2-5% in patients with liver cirrhosis.

Three presumed mechanisms have been proposed:

  • vasoactive substances (e.g. serotonin, interleukin 1, endothelin 1, and thromboxane) cause vasoconstriction and mitogenesis in pulmonary arteries, bypass the liver through portosystemic shunts or not effectively cleared by the diseased cirrhotic liver
  • venous thromboembolism: blood clots from the portal vein or other systemic sources passing through portosystemic shunts to reach the pulmonary circulation, causing pulmonary hypertension
  • high cardiac output associated with cirrhosis exposing the pulmonary vascular bed to increased shear stress and in turn causing vasoconstriction, hypertrophy, and the proliferation of pulmonary arterial endothelial cells
Suggested diagnostic criteria
  • mean pulmonary artery pressure greater than 25 mm Hg at rest
  • increased pulmonary vascular resistance, pulmonary capillary wedge pressure less than 15 mmHg
  • evidence of portal hypertension
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Article information

rID: 32236
Section: Pathology
Synonyms or Alternate Spellings:
  • Porto-pulmonary hypertension
  • Portal-pulmonary hypertension
  • Portopulmonary hypertension (POPH)
  • PPHTN
  • Porto-pulmonary hypertension (PPHTN)
  • Portopulmonary hypertension (PPHTN)

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