Liver transplantation for Budd-Chiari syndrome: a retrospective study

A retrospective study was performed on 11 patients who underwent orthotopic liver transplantation for Budd-Chiari syndrome (BCS), 3 of whom had fulminant type BCS and 8, chronic type BCS. Both the 3- and 5-year actuarial survival rates were 64%, after one patient with fulminant, and three with chronic disease died of sepsis or multiple organ failure following transplantation. Anticoagulation therapy in the early postoperative period was tailored to each individual patient. Most of the patients received heparin for several days and were then converted to Coumadin therapy, although some were not given heparin in the immediate postoperative period but were instead commenced on oral Coumadin after the prothrombin time had recovered to within the normal range. All the long-term survivors had received Coumadin therapy and there was no recurrence of BCS and no early thrombotic or hemorrhagic event. One patient developed late thrombosis of the portal vein despite having received apparently adequate Coumadin therapy. It was thus concluded that liver transplantation is an effective therapy for both fulminant and chronic BCS, and that immediate postoperative heparinization is not mandatory for all patients.