Endothelin-receptor antagonist treatment of portopulmonary hypertension.

Background & Aims: Portal hypertension leading to variceal bleeding and ascites is a severe complication of liver cirrhosis and is associated with a high mortality rate. Endogenous vasoconstrictors including endothelin might play a role in control of intrahepatic vascular tone. Few patients develop pulmonary hypertension in the setting of portal hypertension, known as portopulmonary hypertension.