Budd-Chiari syndrome medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2]

Overview

Medical therapy can be used for short-term symptomatic relief in Budd-Chiari syndrome. However, the use of such medical therapy alone is associated with a high 2-year mortality rate. A low-sodium diet is recommended for patients with Budd-Chiari syndrome. This helps in effective control of ascites. Symptomatic treatment includes diuretics to control ascites, anticoagulants such as heparin and warfarin for hypercoagulable state and antifibrinolytic agents.

Medical Therapy

• The therapy for Budd-Chiari syndrome is aimed at alleviating the obstruction. Underlying conditions are aggressively treated.
• Patients with Budd-Chiari syndrome are treated according to the severity of the disease. Treatment options include:
  • Initial medical therapy
  • Endovascular procedure to restore vessel patency include:
    • Angioplasty
    • Stenting
    • Local thrombolysis
  • Transjugular intrahepatic portosystemic shunt (TIPS)
  • Liver transplantation

Medical therapy

• Medical therapy can be used for short-term symptomatic relief. However, the use of such medical therapy alone is associated with a high 2-year mortality rate.
• A low-sodium diet is recommended for patients of Budd-Chiari syndrome. This helps in effective control of ascites.
• Symptomatic treatment includes diuretics to control ascites, anticoagulants such as heparin and warfarin for hypercoagulable state and antifibrinolytic agents.

Anticoagulation

• Anticoagulation is recommended in all patients of BCS to prevent progression of the thrombosis.
• Anticoagulation with low molecular weight heparin LMWH should be initiated without delay soon after diagnosis. The risk of associated bleeding complications is comparable to patients with anticoagulation therapy for other indications.
• Anticoagulation is maintained with a target value of Anti-Xa between 0.5 and 0.8 IU/ml.
• The goal is to maintain INR between 2.5 and 3 monitored by regular INR testing.
• Prothrombin time and activated partial thromboplastin time are measured once anticoagulation is started and should be maintained within the therapeutic range.
• Before switching from low molecular weight heparin (LMWH) to oral anticoagulants, all contraindications have to be ruled out and a complete diagnostic workup has to be completed.

Thrombolysis

• Thrombolytic agents include streptokinase, urokinase, recombinant tissue-type plasminogen activator (rt-PA).
• Local thrombolysis performed by an interventional radiologist is preferable over systemic thrombolysis.
• Systemic or intra-arterial thrombolysis in BCS has to be administered locally into the hepatic vein, inferior vena cava, and TIPS in case of acute thrombosis.