[Fibrinolytic and anticoagulation therapy in Budd-Chiari syndrome. Review and recommendations]

In Western countries, hepatic vein thrombosis (Budd-Chiari syndrome) is, as a rule, an uncommon though well-known complication of acquired or hereditary thrombophilia. It may be complicated by overt defibrination when secondary to deep venous thrombosis ascending to the vena cava. To be effective treatment should consist of early initial intensive thrombolysis for up to one week, followed without interruption by an APTT-controlled heparin infusion. Oral anticoagulant therapy is started together with the heparin, which should not be discontinued until the prothrombin times, expressed as international normalized ratios (INR), lie within a therapeutic range of 3-5 INR for 2 days, the target being 4 INR. After this intensity of anticoagulation has been maintained for three weeks, the range may be lowered to 2.5-4 INR, the target being 3 INR. Oral anticoagulation should be continued for as long as thrombophilia persists.