Hepatic transplantation with perioperative and long term anticoagulation as treatment for Budd-Chiari syndrome.
Conventional medical and surgical management of Budd-Chiari syndrome is often unsuccessful. In this communication, we report the results of 19 hepatic transplants done for 17 patients suffering from Budd-Chiari syndrome. The first patient who had a transplant did not receive anticoagulant therapy during the postoperative period, and recurrent thrombosis of the hepatic veins in the newly transplanted liver rapidly developed. Sixteen patients who subsequently underwent transplantation were managed using a philosophy of early postoperative anticoagulant treatment when feasible. Using this approach, the cumulative proportion of patients surviving at one and three years was 88 per cent, and no recurrent hepatic vein thrombosis occurred during a mean follow-up period of 28.2 months. Forty-four per cent of the patients who were deliberately given anticoagulant medication experienced a hemorrhagic complication in the postoperative period, but there were no associated deaths. In spite of anticoagulant therapy, 31 per cent of the patients on anticoagulantion medication experienced a thrombotic complication that did not involve the hepatic veins; one early and one late death resulted, and a third patient required urgent retransplantation. Despite the difficulties in patient management, a carefully selected population of patients with hepatic failure secondary to Budd-Chiari syndrome appear to be well served by hepatic transplantation with early postoperative and long term anticoagulant therapy.