Publicaciones científicas

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

15-feb-2024 | Revista: Hepatology

Edo Dongelmans  1 , Nicole Erler  2 , Rene Adam  3 , Silvio Nadalin  4 , Vincent Karam  3 , Sezai Yilmaz  5 , Claire Kelly  6 , Jacques Pirenne  7 , Koray Acarli  8 , Michael Allison  9 , Abdul Hakeem  10 , Dhakshinamoorthy Vijayanand  10 , Dzmitry Fedaruk  11 , Oleg Rummo  11 , Murat Kilic  12 , Arno Nordin  13 , Lutz Fischer  14 , Alessandro Parente  15 , Darius Mirza  15 , William Bennet  16 , Yaman Tokat  17 , Francois Faitot  18 , Barbara B Antonelli  19 , Gabriela Berlakovich  20 , David Patch  21 , Frederik Berrevoet  22 , Marija Ribnikar  23 , Theophile Gerster  24 , Eric Savier  25 , Salvatore Gruttadauria  26   27 , Bo-Göran Ericzon  28 , Andrés Valdivieso  29 , Valentin Cuervas-Mons  30 , Baltasar Perez Saborido  31 , Roland S Croner  32 , Luciano De Carlis  33 , Giulia Magini  34 , Roberta Rossi  35 , Irinel Popescu  36 , Laze Razvan  36 , Stefan Schneeberger  37 , Hans Blokzijl  38 , Laura Llado  39 , Miguel Angel Gomez Bravo  40 , Christophe Duvoux  41 , Vladimír Mezjlík  42 , Gabriel C Oniscu  43 , Kelsey Pearson  43 , Murat Dayangac  44 , Valerio Lucidi  45 , Olivier Detry  46 , Fernando Rotellar  47 , Caroline den Hoed  1 , Wojciech G Polak  48 , Sarwa Darwish Murad  1 ; all other contributing centers (www.eltr.org) for the European Liver and Intestine Transplant Association (ELITA)


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.

CITA DEL ARTÍCULO  Hepatology. 2024 Feb 15.  doi: 10.1097/HEP.0000000000000778