Budd-Chiari Syndrome: a Clinical Approach

Budd-Chiari syndrome is characterized by supra-hepatic veins obstruction, leading to post-sinusoidal portal hypertension that often evolves to hepatic failure. It is usually related to prothrombotic conditions, such as trombophilia, myeloproliferative diseases or nocturnal paroxysmal hemoglobinuria. Spontaneous remissions are rare and less than a third of the patients survive one year without treatment. We recommend that anticoagulation should be started as soon as possible with full-dose subcutaneous heparin, postponing warfarin therapy until substantial improvement of ascites and liver congestion. This approach optimizes anticoagulation, decreasing the chances of bleeding. Since January 2000, among 350 patients followed at the Anticoagulation Clinic, three fulfilled the criteria for primary Budd-Chiari syndrome and were started on scheduled anticoagulation protocol. During three to ten years follow-up, supra-hepatic thrombosis completely resolved in all patients and hepatic function normalized without resorting to invasive procedures or liver transplantation. Neither recurrence of thrombotic events, nor serious bleeding events were documented. Scheduled anticoagulation is safe and improves patient’s outcomes.