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

Edo Dongelmans; Nicole Erler; Rene Adam; Silvio Nadalin; Vincent Karam; Sezai Yilmaz; Claire Kelly; Jacques Pirenne; Koray Acarli; Michael Allison; Abdul Hakeem; Vijayanand Dhakshinamoorthy; Dzmitry Fedaruk; Oleg Rummo; Murat Kilic; Arno Nordin; Lutz Fischer; Alessandro Parente; Darius Mirza; William Bennet; Yaman Tokat; Francois Faitot; Barbara B. Antonelli; Gabriela Berlakovich; David Patch; Frederik Berrevoet; Marija Ribnikar; Theophile Gerster; Eric Savier; Salvatore Gruttadauria; Bo Göran Ericzon; Andrés Valdivieso; Valentin Cuervas-Mons; Baltasar Perez Saborido; Roland S. Croner; Luciano De Carlis; Giulia Magini; Roberta Rossi; Irinel Popescu; Laze Razvan; Stefan Schneeberger; Hans Blokzijl; Laura Llado; Miguel Angel Gomez Bravo; Christophe Duvoux; Vladimír Mezjlík; Gabriel C. Oniscu; Kelsey Pearson; Murat Dayangac; Valerio Lucidi; Olivier Detry; Fernando Rotellar; Caroline Den Hoed; Wojciech G. Polak; Sarwa Darwish Murad

doi:10.1097/HEP.0000000000000778

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

Edo Dongelmans, Nicole Erler, Rene Adam, Silvio Nadalin, Vincent Karam, Sezai Yilmaz, Claire Kelly, Jacques Pirenne, Koray Acarli, Michael Allison, Abdul Hakeem, Vijayanand Dhakshinamoorthy, Dzmitry Fedaruk, Oleg Rummo, Murat Kilic, Arno Nordin, Lutz Fischer, Alessandro Parente, Darius Mirza, William BennetYaman Tokat, Francois Faitot, Barbara B. Antonelli, Gabriela Berlakovich, David Patch, Frederik Berrevoet, Marija Ribnikar, Theophile Gerster, Eric Savier, Salvatore Gruttadauria, Bo Göran Ericzon, Andrés Valdivieso, Valentin Cuervas-Mons, Baltasar Perez Saborido, Roland S. Croner, Luciano De Carlis, Giulia Magini, Roberta Rossi, Irinel Popescu, Laze Razvan, Stefan Schneeberger, Hans Blokzijl, Laura Llado, Miguel Angel Gomez Bravo, Christophe Duvoux, Vladimír Mezjlík, Gabriel C. Oniscu, Kelsey Pearson, Murat Dayangac, Valerio Lucidi, Olivier Detry, Fernando Rotellar, Caroline Den Hoed, Wojciech G. Polak, Sarwa Darwish Murad^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

1 Citation (Scopus)

Abstract

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 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 (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 supplemental 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 (PVT) occurred in 9% and 7%, while recurrent BCS was rare (3%). Conclusions: 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. 2024 American Association for the Study of Liver Diseases. Published by Wolters Kluwer Health, Inc.

Original language	English
Pages (from-to)	136-151
Number of pages	16
Journal	Hepatology
Volume	80
Issue number	1
DOIs	https://doi.org/10.1097/HEP.0000000000000778
Publication status	Published - Jul 2024

Bibliographical note

Publisher Copyright:
© 2024 American Association for the Study of Liver Diseases. Published by Wolters Kluwer Health, Inc.

Access to Document

10.1097/HEP.0000000000000778

Cite this

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., ... Darwish Murad, S. (2024). Recent outcomes of liver transplantation for Budd-Chiari syndrome: A study of the European Liver Transplant Registry (ELTR) and affiliated centers. Hepatology, 80(1), 136-151. https://doi.org/10.1097/HEP.0000000000000778

@article{0737279bd31e46f0aa1532048e44a212,

title = "Recent outcomes of liver transplantation for Budd-Chiari syndrome: A study of the European Liver Transplant Registry (ELTR) and affiliated centers",

abstract = "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 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 (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 supplemental 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 (PVT) occurred in 9% and 7%, while recurrent BCS was rare (3%). Conclusions: 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. 2024 American Association for the Study of Liver Diseases. Published by Wolters Kluwer Health, Inc.",

author = "Edo Dongelmans and Nicole Erler and Rene Adam and Silvio Nadalin and Vincent Karam and Sezai Yilmaz and Claire Kelly and Jacques Pirenne and Koray Acarli and Michael Allison and Abdul Hakeem and Vijayanand Dhakshinamoorthy and Dzmitry Fedaruk and Oleg Rummo and Murat Kilic and Arno Nordin and Lutz Fischer and Alessandro Parente and Darius Mirza and William Bennet and Yaman Tokat and Francois Faitot and Antonelli, {Barbara B.} and Gabriela Berlakovich and David Patch and Frederik Berrevoet and Marija Ribnikar and Theophile Gerster and Eric Savier and Salvatore Gruttadauria and Ericzon, {Bo G{\"o}ran} and Andr{\'e}s Valdivieso and Valentin Cuervas-Mons and {Perez Saborido}, Baltasar and Croner, {Roland S.} and {De Carlis}, Luciano and Giulia Magini and Roberta Rossi and Irinel Popescu and Laze Razvan and Stefan Schneeberger and Hans Blokzijl and Laura Llado and {Gomez Bravo}, {Miguel Angel} and Christophe Duvoux and Vladim{\'i}r Mezjl{\'i}k and Oniscu, {Gabriel C.} and Kelsey Pearson and Murat Dayangac and Valerio Lucidi and Olivier Detry and Fernando Rotellar and {Den Hoed}, Caroline and Polak, {Wojciech G.} and {Darwish Murad}, Sarwa",

note = "Publisher Copyright: {\textcopyright} 2024 American Association for the Study of Liver Diseases. Published by Wolters Kluwer Health, Inc.",

year = "2024",

month = jul,

doi = "10.1097/HEP.0000000000000778",

language = "English",

volume = "80",

pages = "136--151",

journal = "Hepatology",

issn = "0270-9139",

publisher = "John Wiley & Sons Ltd.",

number = "1",

}

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 2024, 'Recent outcomes of liver transplantation for Budd-Chiari syndrome: A study of the European Liver Transplant Registry (ELTR) and affiliated centers', Hepatology, vol. 80, no. 1, pp. 136-151. https://doi.org/10.1097/HEP.0000000000000778

TY - JOUR

T1 - Recent outcomes of liver transplantation for Budd-Chiari syndrome

T2 - A study of the European Liver Transplant Registry (ELTR) and affiliated centers

AU - Dongelmans, Edo

AU - Erler, Nicole

AU - Adam, Rene

AU - Nadalin, Silvio

AU - Karam, Vincent

AU - Yilmaz, Sezai

AU - Kelly, Claire

AU - Pirenne, Jacques

AU - Acarli, Koray

AU - Allison, Michael

AU - Hakeem, Abdul

AU - Dhakshinamoorthy, Vijayanand

AU - Fedaruk, Dzmitry

AU - Rummo, Oleg

AU - Kilic, Murat

AU - Nordin, Arno

AU - Fischer, Lutz

AU - Parente, Alessandro

AU - Mirza, Darius

AU - Bennet, William

AU - Tokat, Yaman

AU - Faitot, Francois

AU - Antonelli, Barbara B.

AU - Berlakovich, Gabriela

AU - Patch, David

AU - Berrevoet, Frederik

AU - Ribnikar, Marija

AU - Gerster, Theophile

AU - Savier, Eric

AU - Gruttadauria, Salvatore

AU - Ericzon, Bo Göran

AU - Valdivieso, Andrés

AU - Cuervas-Mons, Valentin

AU - Perez Saborido, Baltasar

AU - Croner, Roland S.

AU - De Carlis, Luciano

AU - Magini, Giulia

AU - Rossi, Roberta

AU - Popescu, Irinel

AU - Razvan, Laze

AU - Schneeberger, Stefan

AU - Blokzijl, Hans

AU - Llado, Laura

AU - Gomez Bravo, Miguel Angel

AU - Duvoux, Christophe

AU - Mezjlík, Vladimír

AU - Oniscu, Gabriel C.

AU - Pearson, Kelsey

AU - Dayangac, Murat

AU - Lucidi, Valerio

AU - Detry, Olivier

AU - Rotellar, Fernando

AU - Den Hoed, Caroline

AU - Polak, Wojciech G.

AU - Darwish Murad, Sarwa

PY - 2024/7

Y1 - 2024/7

N2 - 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 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 (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 supplemental 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 (PVT) occurred in 9% and 7%, while recurrent BCS was rare (3%). Conclusions: 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. 2024 American Association for the Study of Liver Diseases. Published by Wolters Kluwer Health, Inc.

AB - 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 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 (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 supplemental 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 (PVT) occurred in 9% and 7%, while recurrent BCS was rare (3%). Conclusions: 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. 2024 American Association for the Study of Liver Diseases. Published by Wolters Kluwer Health, Inc.

UR - http://www.scopus.com/inward/record.url?scp=85196532463&partnerID=8YFLogxK

U2 - 10.1097/HEP.0000000000000778

DO - 10.1097/HEP.0000000000000778

M3 - Article

C2 - 38358658

AN - SCOPUS:85196532463

SN - 0270-9139

VL - 80

SP - 136

EP - 151

JO - Hepatology

JF - Hepatology

IS - 1

ER -

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

Abstract

Bibliographical note

Access to Document

Other files and links

Fingerprint

Cite this