Orthotopic Liver Transplant for Budd-Chiari Syndrome: An Analysis of 14 Cases.

OBJECTIVES Budd-Chiari syndrome is a low-prevalence, life-threatening disorder characterized by hepatic venous outflow obstruction at the hepatic venules, the large hepatic veins, the inferior vena cava, or the right atrium. Orthotopic liver transplant should be considered for patients with fulminant and chronic forms of the syndrome. MATERIALS AND METHODS Fourteen patients received 15 orthotropic liver transplants at our center from September 2006 to March 2013. This study retrospectively reviewed the prospectively collected data from these 14 patients. RESULTS The mean age of the patients was 33 years; only 1 patient was female. The severity of liver disease was Child-Pugh score A in 1 patient, B in 4 patients, and C in 9 patients. Mean calculated Model for End-Stage Liver Disease score was 18 (range, 6-30). The cause of Budd-Chiari syndrome was factor 5 Leiden mutation in 3 patients, polycythemia vera in 2 patients, factor 2 and 3 deficiency in 1 patient, fulminant essential thrombocytosis in 1 patient, and protein C deficiency in 2 patients. We performed 15 transplants in 14 patients. Five grafts were obtained from deceased donors, and 10 grafts were from living-related donors. Mean graft-to-recipient weight ratio was 1,12 for patients receiving a living-donor liver transplant. Median follow-up was 29 months. Patient survival rates were 87%, 71%, and 71% at 1, 3, and 5 years. CONCLUSIONS Liver transplant is an option for treating Budd-Chiari syndrome in cases of fulminant presentation and cirrhosis. Living-donor liver transplant is a viable choice in countries where procuring organ donations is still a problem. To manage the long-term medical therapy and follow-up for these patients, a careful evaluation is necessary to determine the cause of Budd-Chiari syndrome. Anticoagulant and antiaggregant therapy remains the mainstay of treatment for this syndrome.