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Experience of Liver Transplant in Patients Diagnosed with Budd-Chiari Syndrome
Syed Muhammad Raza1, Saima Zainab, Ali Reza Shamsaeefar
1From the Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
Insights
Liver transplantation offers a successful treatment for advanced Budd-Chiari syndrome. Post-transplant anticoagulation therapy significantly improves patient survival rates in these complex cases.
Area of Science:
- Hepatology
- Transplant Surgery
- Vascular Surgery
Background:
- Budd-Chiari syndrome can progress to severe liver damage, including cirrhosis and fulminant hepatic failure.
- Treatment strategies for Budd-Chiari syndrome are contingent upon disease severity.
- Liver transplantation is a recognized and effective therapeutic option for patients with advanced-stage Budd-Chiari syndrome.
Purpose of the Study:
- To evaluate the outcomes of liver transplantation in patients diagnosed with Budd-Chiari syndrome.
- To assess the long-term survival rates and complications associated with liver transplantation for Budd-Chiari syndrome.
Main Methods:
- A retrospective analysis of liver transplants performed for Budd-Chiari syndrome between 1993 and January 2016.
- Inclusion criteria encompassed all patients undergoing liver transplantation for Budd-Chiari syndrome at Shiraz University of Medical Sciences.
- Data collected included patient demographics, pre-transplant interventions, graft types, and post-transplant outcomes.
Main Results:
- A total of 68 liver transplants for Budd-Chiari syndrome were identified from 3201 procedures.
- The cohort comprised 27 males and 41 females with a median age of 31 years.
- The five-year survival rate was 89%, with 6 deaths occurring post-transplant, including one re-transplantation due to disease recurrence.
Conclusions:
- Liver transplantation, when combined with post-transplant anticoagulation therapy, demonstrably enhances survival for individuals with advanced Budd-Chiari syndrome.
- This approach offers a viable solution for managing the severe sequelae of Budd-Chiari syndrome.
- Further research into optimizing anticoagulation protocols may further improve outcomes.
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.
Materials 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 pretransplant interventions, with 2 treated with inferior vena cava stenting and 3 having transjugular intrahepatic 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 posttransplant anticoagulation therapy can improve the survival of patients with advanced-stage Budd-Chiari syndrome.
