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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Overview
A minority of patients can be treated medically with sodium restriction, diuretics to control ascites, anticoagulants such as heparin and warfarin, and general symptomatic management. The majority of patients require further intervention. Milder forms of Budd-Chiari may be treated with surgical shunts to divert blood flow around the obstruction or the liver itself. Shunts must be placed early after diagnosis for best results. The transjugular intrahepatic portosystemic shunt (TIPS) is similar to a surgical shunt. It accomplishes the same goal but has a lower procedure-related mortality, which has led to a growth in its popularity. Patients with stenosis or vena caval obstruction may benefit from angioplasty. [1] Limited studies on thrombolysis with direct infusion of urokinase and tissue plasminogen activator (tPA) into the obstructed vein have shown moderate success in treating Budd-Chiari syndrome; however, it is not routinely attempted.
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 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.
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.
References
- ↑ Fisher NC, McCafferty I, Dolapci M, Wali M, Buckels JA, Olliff SP, Elias E. Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut. 1999 Apr;44(4):568-74.