Budd-Chiari syndrome

Budd-Chiari syndrome (BCS) is a rare vascular disorder characterized by obstruction of hepatic venous outflow, occurring at the level of the hepatic venules, the large hepatic veins, or the inferior vena cava. This obstruction impedes blood drainage from the liver, leading to hepatic congestion, portal hypertension, and progressive liver damage. The condition may also be referred to as hepatic vein thrombosis. BCS can present in acute, subacute, or chronic forms. Acute presentations include sudden onset of severe abdominal pain, hepatomegaly (enlarged liver), ascites (fluid accumulation in the abdomen), and jaundice. Chronic forms may develop insidiously with progressive ascites, variceal bleeding from portal hypertension, and eventual liver cirrhosis and hepatic failure. The underlying cause of Budd-Chiari syndrome is most often related to a prothrombotic (blood clot–promoting) condition. Myeloproliferative neoplasms, particularly polycythemia vera and essential thrombocythemia, are among the most common predisposing factors. Other associated conditions include antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria, inherited thrombophilias (such as Factor V Leiden mutation or protein C/S deficiency), oral contraceptive use, pregnancy, and certain infections or malignancies. In some cases, a membranous obstruction of the inferior vena cava is identified, which is more prevalent in Asian and African populations. Treatment of Budd-Chiari syndrome is stepwise and depends on the severity and acuity of the disease. Anticoagulation therapy is the cornerstone of initial management to prevent clot propagation. If anticoagulation alone is insufficient, interventional procedures such as angioplasty, stenting of the hepatic veins or inferior vena cava, or transjugular intrahepatic portosystemic shunt (TIPS) placement may be performed to restore hepatic venous outflow. In cases of fulminant hepatic failure or end-stage liver disease refractory to other treatments, liver transplantation remains a definitive therapeutic option. Early diagnosis and a multidisciplinary approach involving hepatologists, hematologists, and interventional radiologists are essential for optimizing outcomes.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Budd-Chiari syndrome