Liver and Bile

Hepatology. 2024;80(1):136–51

Dongelmans E, Erler N, Adam R, Nadalin S, Karam V, Yilmaz S, Kelly C, Pirenne J, Acarli K, Allison M, Hakeem A, Dhakshinamoorthy V, Fedaruk D, Rummo O, Kilic M, Nordin A, Fischer L, Parente A, Mirza D, Bennet W, Tokat Y, Faitot F, Antonelli BB, Berlakovich G, Patch D, Berrevoet F, Ribnikar M, Gerster T, Savier E, Gruttadauria S, Ericzon BG, Valdivieso A, Cuervas-Mons V, Perez Saborido B, Croner RS, De Carlis L, Magini G, Rossi R, Popescu I, Razvan L, Schneeberger S, Blokzijl H, Llado L, Gomez Bravo MA, Duvoux C, Mezjlík V, Oniscu GC, Pearson K, Dayangac M, Lucidi V, Detry O, Rotellar F, den Hoed C, Polak WG, Darwish Murad S; European Liver and Intestine Transplant Association (ELITA)

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 (post-LT) outcomes in Europe.
Approach and results: Data from all patients who underwent transplantation from 1976 to 2020 was obtained from the European Liver Transplant Registry (ELTR). Patients < 16 years, with secondary BCS or hepatocellular carcinoma (HCC) were excluded. Patient survival (PS) and graft survival (GS) before and after 2000 were compared. Multivariate Cox regression analysis identified predictors of PS and GS after 2000. Supplemental data was requested from all ELTR-affiliated centers and received from 44. In all, 808 patients underwent transplantation between 2000 and 2020. One-, 5- and 10-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 retransplantation rate was 12%. Recipient age (adjusted hazard ratio [aHR] = 1.04, 95% confidence interval [CI]: 1.02–1.06) and Model for End-stage Liver Disease (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 supplemental data, 33% had myeloproliferative neoplasm, 20% underwent placement of a transjugular intrahepatic portosystemic shunt (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%).

Conclusions: Liver transplantation for Budd-Chiari syndrome results in excellent patient and graft survival. Older recipient or donor age and higher Model for End-stage Liver Disease (MELD) score are associated with poorer outcomes, while long-term anticoagulation improves both patient and graft outcomes.

DOI:  10.1097/hep.0000000000000778