Timing of Liver Transplantation FollowingTransjugular Intrahepatic PortosystemicShunt in Patients with Budd-ChiariSyndrome

摘要

Background Aims: Budd-Chiari syndrome (BCS) is a rare and life-threatening disorder secondary to he-patic venous outflow obstruction. Small series of BCSpatients indicate that transjugular intrahepatic porto-systemic shunt (TIPS) may be useful. However, theinfluence of TIPS on patient survival and factors thatpredict the outcome of TIPS in BCS patients remainunknown. Methods: One hundred twenty-four consec-utive BCS patients treated with TIPS in 6 Europeancenters between July 1993 and March 2006 were fol-lowed until death, orthotopic liver transplantation(OLT), or last clinical evaluation. Results: Prior totreatment with TIPS, BCS patients had a high Model ofEnd Stage Liver Disease and high Rotterdam BCSprognostic index (98% of patients at intermediate orhigh risk) indicating severity of liver dysfunction.However, 1- and 5-year OLT-free survival were 88% and78%, respectively. In the high-risk patients, 5-yearOLT-free survival was much better than that estimatedby the Rotterdam BCS index (71% vs 42%, respec-tively). In the whole population, bilirubin, age, andinternational normalized ratio for prothrombin timeindependently predicted 1-year OLT-free survival. Aprognostic score with a good discriminative capacity(area under the curve, 0.86) was developed from thesevariables. Seven out of 8 patients with a score >7 diedor underwent transplantation vs 5 out of 114 patientswith a score <7. Conclusions: Long-term outcome forpatients with severe BCS treated with TIPS is excellenteven in high-risk patients, suggesting that TIPS mayimprove survival. Furthermore, we identified a smallsubgroup of BCS patients with poor prognosis despiteTIPS who might benefit from early OLT.