Abstract
Budd-Chiari syndrome (BCS) is a rare but fatal disease caused by the obstruction in hepatic venous outflow tract (usually by thrombosis) and is further classified into two subtypes depending on the level of obstruction. Patients with BCS often have a combination of prothrombotic risk factors. Clinical presentation is diverse. Stepwise management strategy has been suggested with excellent 5-year survival rate. It includes anticoagulation, treatment of identified prothrombotic risk factor, percutaneous recanalization, and transjugular intrahepatic portosystemic shunt (TIPS) to reestablish hepatic venous outflow and liver transplantation in unresponsive patients. Owing to the rarity of BCS, there are no randomized controlled trials (RCTs) precisely identifying the timing for TIPS. TIPS should be considered in patients with refractory ascites, variceal bleed, and fulminant liver failure. Liver replacement is indicated in patients with progressive liver failure and in those in whom TIPS is not technically possible. The long-term outcome is usually influenced by the underlying hematologic condition and the development of hepatocellular carcinoma. This review focuses on the timing and the long-term efficacy of TIPS in patients with BCS.