Successful liver transplantation in a patient with Budd-Chiari syndrome caused by homozygous factor V Leiden

Budd-Chiari syndrome (BCS) is a rare form of portal hypertension characteri zed by hepatic venous outflow obstruction. Although hematologic disorders a re the most common cause of this syndrome, to date, 30% of the cases have b een classified as idiopathic. Resistance to activated protein C caused by f actor V Leiden is the most common cause of thrombophilia; its role in the p athogenesis of BCS is now becoming apparent. Wk report successful liver tra nsplantation in a patient with BCS caused by homozygous factor V Leiden. Th e patient was administered standard heparin anticoagulation until activated protein C resistance was normalized by the liver allograft. Liver transpla ntation corrected the thrombophilic state. The patient has excellent graft function, is not on anticoagulation therapy, and has had no recurrent venou s thrombosis at 5 months posttransplantation. Activated protein C resistanc e caused by the factor V Leiden mutation may be responsible for idiopathic cases of BCS. To avoid unnecessary long-term anticoagulation after liver tr ansplantation, factor V Leiden should be considered as a pathogenic factor in BCS. In addition, because of the high prevalence of factor V Leiden in t he world population, cadaveric organ donors with a history of venous thromb osis should be screened for activated protein C resistance lest thrombophil ia be transmitted to the recipient.