Experience of Liver Transplant in Patients Diagnosed with Budd-Chiari Syndrome

Objectives: Budd-Chiari syndrome can lead to fulminant hepatic failure and cirrhosis. The treatment depends on the severity of disease. Liver transplant is a successful treatment option for those with advanced-stage disease. Material and Methods: In this retrospective study, we analyzed all liver transplants conducted for Budd-Chiari syndrome at the organ transplant unit of Shiraz University of Medical Sciences, Iran, from 1993 to January 2016. Overall, 3201 liver transplant procedures were performed. Among these, 68 presented with Budd-Chiari syndrome. Results: The median age was 31 years among 27 male and 41 female patients. Five patients received pre-transplant interventions, with 2 treated with inferior vena cava stenting and 3 having transjugular intra-hepatic portosystemic shunts. Sixty-five patients with Budd-Chiari syndrome received deceased-donor grafts and 3 received living-donor grafts. Among the Budd-Chiari transplant patients, 6 patients died. Five deaths occurred in the early posttransplant period, and 1 patient retransplanted after 2 years for recurrence of disease died due to graft failure. The five-year survival rate was 89% among patients with Budd-Chiari syndrome. Conclusions: Liver transplant along with post transplant anticoagulation therapy can improve the survival of patients with advanced-stage Budd-Chiari syndrome.