Budd-Chiari syndrome medical therapy: Difference between revisions

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==Overview==
==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]]. <ref>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.</ref> 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==
==Medical Therapy==
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===Medical Therapy===
===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.
*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===
*Anticoagulation is recommended in all patients of BCS to prevent progression of the thrombosis.
*Anticoagulation is recommended in all patients of BCS to prevent progression of the thrombosis.

Revision as of 19:59, 9 November 2017

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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

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.

References