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Multicenter Study
. 2023 Jun 1;107(6):1330-1340.
doi: 10.1097/TP.0000000000004444. Epub 2023 May 23.

Liver Transplantation for Porto-sinusoidal Vascular Liver Disorder: Long-term Outcome

Marta Magaz  1   2   3 , Heloïse Giudicelli-Lett  3   4 , Oana Nicoară-Farcău  1   2   3   5 , Neil Rajoriya  6 , Ashish Goel  6 , Karlien Raymenants  7 , Sophie Hillaire  3   4 , Gonzalo Crespo  8   9 , Luis Téllez  10 , Laure Elkrief  11   12 , Constantino Fondevila  13 , Lara Orts  1   2   3 , Filipe Nery  14 , Akash Shukla  15 , Hélène Larrue  16 , Yiliam Fundora  13 , Helena Degroote  17 , Victoria Aguilera  18   19 , Elba LLop  20 , Laura Turco  21 , Federica Indulti  21 , Stefania Gioia  22 , Giulia Tosetti  23 , Niccolò Bitto  23 , Chiara Becchetti  24 , Edilmar Alvarado  25   26 , Cristina Roig  25   26 , Raquel Diaz  27 , Michael Praktiknjo  28 , Anna-Lena Konicek  28 , Guillem Soy  1   2   3 , Pol Olivas  1   2   3 , José Ignacio Fortea  29 , Helena Masnou  30   31 , Ángela Puente  29 , Alba Ardèvol  30   31 , Carmen Álvarez-Navascués  32 , Marta Romero  33 , Bernhard Scheiner  34 , Georg Semmler  34 , Mattias Mandorfer  34 , Filipe Damião  35 , Anna Baiges  1   2   3 , Fanny Turon  1   2   3 , Macarena Simón-Talero  36 , Carlos González-Alayón  37 , Alba Díaz  38 , Ángeles García-Criado  39 , Andrea de Gottardi  40 , Enric Reverter  26   41   42 , Annabel Blasi  43 , Joan Genescà  36 , Olivier Roux  3   4 , Claire Francoz  3   4 , Carlos Noronha Ferreira  35 , Thomas Reiberger  34 , Manuel Rodríguez  32 , Rosa María Morillas  30   31 , Javier Crespo  29 , Jonel Trebicka  44   45 , Rafael Bañares  27 , Càndid Villanueva  25   26 , Annalisa Berzigotti  24 , Massimo Primignani  23 , Vincenzo La Mura  23 , Oliviero Riggio  22 , Filippo Schepis  21 , Bogdan Procopet  5 , Xavier Verhelst  17 , José Luis Calleja  20 , Christophe Bureau  16 , Agustín Albillos  10 , Frederik Nevens  7 , Virginia Hernández-Gea  1   2   3 , Dhiraj Tripathi  6 , Pierre-Emmanuel Rautou  3   4 , François Durand  3   4 , Juan Carlos García-Pagán  1   2   3
Affiliations
Multicenter Study

Liver Transplantation for Porto-sinusoidal Vascular Liver Disorder: Long-term Outcome

Marta Magaz et al. Transplantation. .

Abstract

Background: Porto-sinusoidal vascular liver disorder (PSVD) is a rare disease that occasionally requires liver transplantation (LT), despite usually presenting preserved liver function. There remains a paucity of data pertaining to LT in PSVD. The aim was to identify features associated with post-LT outcomes in PSVD.

Methods: Retrospective multicentre study of 79 patients who received LT for PSVD.

Results: Median post-LT follow-up was 37 (range 1-261) mo. Refractory ascites 24 (30%), hepatic encephalopathy 16 (20%), and hepatopulmonary syndrome 13 (16.3%) were the most frequent indications for LT. Hepatocellular carcinoma was the indication in only 2 patients. Twenty-four patients died, 7 due to liver and 17 to non-liver related causes. Post-LT survival was 82.2%, 80.7%, and 68.6% at 1, 2, and 5 y, respectively. Post-LT survival was significantly better in patients without (n = 58) than in those with a persistent severe PSVD-associated condition (n = 21). Pre-LT hyperbilirubinemia levels and creatinine >100 µmol/L were also independently associated with poor survival. Six patients (7.6%) required a second LT. Recurrence of PSVD was confirmed by liver biopsy in only 1 patient and in 3 further patients it was likely.

Conclusions: LT in PSVD is associated with an acceptable outcome in the absence of associated severe conditions. However, persistence of a severe associated condition, pre-LT high bilirubin levels, or creatinine >100 µmol/L impact outcome, and these are features that should be considered when evaluating PSVD patients for LT. PSVD recurrence is possible after LT and needs to be explored, at least, in cases of posttransplant portal hypertension.

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Conflict of interest statement

V.H.-G. received speaker fees from Gore. J.C.G.-P. advises for GORE, Cook, and Shionogi. The other authors declare no conflicts of interest.

Comment in

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