Obstruction of the main hepatic veins or suprahepatic inferior vena cava is caused mainly by thrombosis or its fibrous sequela. One or several underlying thrombogenic disorders are usually present, the most common of which is an overt or occult primary myeloproliferative disorder. The major complications are ascites and gastrointestinal bleeding. A sizeable proportion of the cases are asymptomatic. Diagnosis is usually made at ultrasound or magnetic resonance imaging when collateral veno-venous circulation or endoluminal hepatic venous material are demonstrated. Natural history is poorly known. Overall, mortality is high in the early phase of the disease but once the acute episode is terminated, prognosis is good. Treatment includes anticoagulation to prevent recurrence or extension of thrombosis, nonspecific measures to control ascites and gastrointestinal bleeding, and procedures aiming to restore hepatic blood outflow. Portal-caval shunts are preferred when possible. In patients with obstructed inferior vena cava, the first choice is percutaneous angioplasty, and the second choice is portal-systemic shunts with the right atrium or superior vena caval circulation. Transplantation is proposed for patients with severe liver failure.

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