Treatment of Budd-Chiari syndrome in a liver transplant unit, the role of transjugular intrahepatic porto-systemic shunt and liver transplantation

BACKGROUND: Budd-Chiari syndrome is an uncommon cause of liver failure usually associated with an underlying hypercoagulable state. AIM: To evaluate current trends in management of Budd-Chiari syndrome at our institution. METHODS: Twenty-two patients with Budd-Chiari syndrome underwent transjugular intrahepatic porto-systemic shunt, liver transplantation, or both in between 1992 and 2001. We analysed underlying diagnosis, medical therapy, complications, follow-up and overall outcomes. RESULTS: Five patients (17%) presented with fulminant liver failure and 17 patients (83%) with new-onset ascites or chronic liver disease. Seventeen patients (74%) underwent transjugular intrahepatic porto-systemic shunt: improvement or stabilization occurred initially in 14 (82%), whereas the other three patients died within a month. At a mean 3 years follow-up eight patients (47%) continued to do well clinically and four have died (23.5%); seven have required transjugular intrahepatic porto-systemic shunt revisions (mean 2.3 interventions), five have experienced transjugular intrahepatic porto-systemic shunt occlusion managed with new transjugular intrahepatic porto-systemic shunt placement and five patients underwent subsequent transplantation. Of the 10 patients who underwent liver transplantation, patient and graft survival are 80% at a mean 5.7 years of follow-up. No patient developed post-transplant Budd-Chiari syndrome. CONCLUSIONS: Transjugular intrahepatic porto-systemic shunt is usually feasible in patients with Budd-Chiari syndrome, and is best suited as a bridge to more timely liver transplantation. Long-term success of transjugular intrahepatic porto-systemic shunt is limited and usually requires revision, placement of a new shunt or liver transplantation. Liver transplantation with chronic anticoagulation offers excellent short- and medium-term patient and graft survival. In our series, there was no recurrence of Budd-Chiari syndrome after liver transplantation.