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Eur J Gastroenterol Hepatol. 2010 Apr;22(4):385-90. doi: 10.1097/MEG.0b013e3283337130.

Portopulmonary hypertension: short review.

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Divisions of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University of Graz, Austria.


Portopulmonary hypertension (PoPH) is an underrecognized complication of portal hypertension, related to cirrhosis and noncirrhotic portal hypertension. PoPH has been found in 5-6% of patients with decompensated liver disease and may adversely affect outcome after liver transplantation. The prevalence of PoPH is unrelated to the severity of liver disease but associated with female sex and underlying autoimmune liver disease. Diagnosis of PoPH is based on screening with Doppler echocardiography and confirmation by right-heart catheterization. Treatment options with proven efficacy in idiopathic pulmonary hypertension include endothelin receptor antagonists, prostanoids, and sildenafil. In PoPH, such targeted treatment was found to be safe in small uncontrolled studies but randomized trials demonstrating its benefit are lacking.

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