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 of the Dana point 2008 pulmonary hypertension classification system.
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Epidemiology
The prevalence of portopulmonary hypertension ranges from 2-5% in patients with liver cirrhosis.
Pathology
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 mmHg at rest
- increased pulmonary vascular resistance, pulmonary capillary wedge pressure less than 15 mmHg
- evidence of portal hypertension
See also
-
pulmonary complications of cirrhosis and portal hypertension
- hepatopulmonary syndrome (HPS)
- portopulmonary hypertension (POPH)
- hepatic hydrothorax (HH)
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