Liver Transplantation for Porto-sinusoidal Vascular Liver Disorder: Long-term Outcome
Authors: Magaz Marta , Giudicelli-Lett Heloïse , Nicoară-Farcău Oana , Rajoriya Neil , Goel Ashish , Raymenants Karlien , Hillaire Sophie , Crespo Gonzalo , Téllez Luis , Elkrief Laure , Fondevila Constantino , Orts Lara , Nery Filipe , Shukla Akash , Larrue Hélène , Fundora Yiliam , Degroote Helena , Aguilera Victoria , LLop Elba , Turco Laura , Indulti Federica , Gioia Stefania , Tosetti Giulia , Bitto Niccolò , Becchetti Chiara , Alvarado Edilmar , Roig Cristina , Diaz Raquel , Praktiknjo Michael , Konicek Anna-Lena , Soy Guillem , Olivas Pol , Fortea José Ignacio , Masnou Helena , Puente Ángela , Ardèvol Alba , Álvarez-Navascués Carmen , Romero Marta , Scheiner Bernhard , Semmler Georg , Mandorfer Mattias , Damião Filipe , Baiges Anna , Turon Fanny , Simón-Talero Macarena , González-Alayón Carlos , Díaz Alba , García-Criado Ángeles , de Gottardi Andrea , Reverter Enric , Blasi Annabel , Genescà Joan , Roux Olivier , Francoz Claire , Noronha Ferreira Carlos , Reiberger Thomas , Rodríguez Manuel , Morillas Rosa María , Crespo Javier , Trebicka Jonel , Bañares Rafael , Villanueva Càndid , Berzigotti Annalisa , Primignani Massimo , La Mura Vincenzo , Riggio Oliviero , Schepis Filippo , Procopet Bogdan , Verhelst Xavier , Calleja José Luis , Bureau Christophe , Albillos Agustín , Nevens Frederik , Hernández-Gea Virginia , Tripathi Dhiraj , Rautou Pierre-Emmanuel , Durand François , García-Pagán Juan Carlos 
Published: December 2022

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.