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:

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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 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.

Anticoagulation

• Anticoagulation is recommended in all patients of BCS to prevent progression of the thrombosis.
• Anticoagulation with 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 LMWH to oral anticoagulants, all contraindications has 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.