Recent outcomes of liver transplantation for Budd Chiari Syndrome - a study of the European Liver Transplant Registry (ELTR) and affiliated centers.

BACKGROUND AND AIMS:Management of Budd-Chiari Syndrome (BCS) has improved over the last decades. The main aim was to evaluate the contemporary post-liver transplant (LT) outcomes in Europe. APPROACH AND RESULTS:Data from all transplanted patients from 1976-2020 was obtained from the European Liver Transplantation Registry (ELTR). Patients<16yrs, with secondary BCS or hepatocellular carcinoma were excluded. Patient- (PS) and graft survival (GS) before and after 2000 were compared. Multivariate Cox regression-analysis identified predictors of PS and GS after 2000. Supplementary data was requested from all ELTR-affiliated centers and received from 44. 808 patients were transplanted between 2000-2020. One-, five- and ten-year PS was 84%, 77% and 68% and GS was 79%, 70% and 62%, respectively. Both significantly improved, compared to outcomes before 2000 (p<0.001). Median follow up was 50 months and re-transplantation rate was 12%. Recipient age (aHR:1.04,95%CI:1.02-1.06) and MELD-score (aHR:1.04,95%CI:1.01-1.06), especially above 30, were associated with worse PS, while male sex had better outcomes (aHR:0.63,95%CI:0.41-0.96). Donor age was associated with worse PS (aHR:1.01,95%CI:1.00-1.03) and GS (aHR:1.02,95%CI:1.01-1.03). In 353 patients (44%) with supplementary data, 33% had myeloproliferative neoplasm, 20% underwent TIPS pre-LT and 85% used anticoagulation post-LT. Post-LT anticoagulation was associated with improved PS (aHR:0.29,95%CI:0.16-0.54) and GS (aHR:0.48,95%CI:0.29-0.81). Hepatic artery thrombosis and portal vein thrombosis occurred in 9% and 7%, while recurrent BCS was rare (3%). CONCLUSION:LT for BCS results in excellent patient- and graft survival. Older recipient or donor age, and higher MELD are associated with poorer outcomes, while long-term anticoagulation improves both patient and graft outcomes.