Liver Transplantation for Porto-Sinusoidal Vascular Liver Disorder: Long-term Outcome.

Fecha de publicación: 09/12/2022 Fecha Ahead of Print: 09/12/2022

Autores de IIS La Fe

Victoria Aguilera Sancho-Tello

Autor

Participantes ajenos a IIS La Fe

Magaz, Marta
Giudicelli-Lett, Heloise
Nicoara-Farcau, Oana
Rajoriya, Neil
Goel, Ashish
Raymenants, Karlien
Hillaire, Sophie
Crespo, Gonzalo
Tellez, Luis
Elkrief, Laure
Fondevila, Constantino
Orts, Lara
Nery, Filipe
Shukla, Akash
Larrue, Helene
Fundora, Yiliam
Degroote, Helena
LLop, Elba
Turco, Laura
Indulti, Federica
Gioia, Stefania
Tosetti, Giulia
Bitto, Niccolo
Becchetti, Chiara
Alvarado, Edilmar
Roig, Cristina
Diaz, Raquel
Praktiknjo, Michael
Konicek, Anna-Lena
Soy, Guillem
Olivas, Pol
Fortea, Jose Ignacio
Masnou, Helena
Puente, Angela
Ardèvol A
Alvarez-Navascues, Carmen
Romero, Marta
Scheiner, Bernhard
Semmler, Georg
Mandorfer, Mattias
Damião F
Baiges, Anna
Turon, Fanny
Simon-Talero, Macarena
Gonzalez-Alayon, Carlos
Diaz, Alba
Garcia-Criado, Angeles
de Gottardi, Andrea
Reverter, Enric
Blasi, Annabel
Genesca, Joan
Roux, Olivier
Francoz, Claire
Noronha Ferreira C
Reiberger, Thomas
Rodriguez, Manuel
Morillas, Rosa Maria
Crespo, Javier
Trebicka, Jonel
Bañares R
Villanueva, Candid
Berzigotti, Annalisa
Primignani, Massimo
La Mura, Vincenzo
Riggio, Oliviero
Schepis, Filippo
Procopet, Bogdan
Verhelst, Xavier
Calleja, Jose Luis
Bureau, Christophe
Albillos, Agustin
Nevens, Frederik
Hernandez-Gea, Virginia
Tripathi, Dhiraj
Rautou, Pierre-Emmanuel
Durand, Francois
Garcia-Pagan, Juan Carlos

Grupos

Hepatología, Cirugia Hepatobiliopancreática y Trasplantes

Leader

Marina Berenguer Haym

Abstract

BACKGROUND AND AIMS: 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.

Datos de la publicación

ISSN/ISSNe:: 0041-1337, 1534-6080
Tipo:: Article
Páginas:: 1330-1340
DOI:: 10.1097/TP.0000000000004444
Factor de Impacto:: 1,407 SCImago ℠
Cuartil:: Q1 SCImago ℠

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Métricas

Filiaciones

Giudicelli-Lett, Heloise:: Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, DHU Unity, Pôle des Maladies de l'Appareil Digestif, Hôpital Beaujon, AP-HP, Clichy, France
Nicoara-Farcau, Oana:: Regional Institute of Gastroenterology and Hepatology "Octavian Fodor," Hepatology Department and "Iuliu Hatieganu" University of Medicine and Pharmacy, 3rd Medical Clinic, Cluj-Napoca, Romania
Rajoriya, Neil:: The Liver Unit, University Hospital Birmingham NHS Foundation Trust, Birmingham, United Kingdom
Goel, Ashish:: The Liver Unit, University Hospital Birmingham NHS Foundation Trust, Birmingham, United Kingdom
Raymenants, Karlien:: Department of Gastroenterology and Hepatology, University Hospital KU Leuven, Leuven, Belgium
Hillaire, Sophie:: Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, DHU Unity, Pôle des Maladies de l'Appareil Digestif, Hôpital Beaujon, AP-HP, Clichy, France
Crespo, Gonzalo:: Liver Transplantation Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain
Tellez, Luis:: Department of Gastroenterology and Hepatology, Hospital Universitario Ramón y Cajal, IRYCIS, CIBERehd, Universidad de Alcalá, Madrid, Spain
Elkrief, Laure:: Service d'Hépato-Gastroentérologie, Hôpitaux Universitaires de Genève, Geneva, Switzerland. Service d'Hépato-Gastroentérologie, CHU de Tours, France

Université de Paris, Centre de recherche sur l'inflammation, Inserm, U1149, CNRS, ERL8252, Paris, France
Fondevila, Constantino:: Department of Surgery, Division of Hepatobiliary and General Surgery, Institut de Malalties Digestives I Metabòliques (IMDiM), Hospital Clínic, University of Barcelona, Barcelona, Spain
Nery, Filipe:: Liver Unit, Centro Hospitalar do Porto, Hospital Sto Antonio, Porto, Portugal
Shukla, Akash:: Department of Gastroenterology, Lokmanya Tilak Municipal General Hospital and Lokmanya Tilak Municipal Medical College, Sion, Mumbai, India
Larrue, Helene:: Department of Hepatology, Rangueil Hospital, CHU Toulouse, University Paul Sabatier of Toulouse, France
Fundora, Yiliam:: Department of Surgery, Division of Hepatobiliary and General Surgery, Institut de Malalties Digestives I Metabòliques (IMDiM), Hospital Clínic, University of Barcelona, Barcelona, Spain
Degroote, Helena:: Department of Gastroenterology and Hepatology, Ghent University Hospital, Ghent, Belgium
Aguilera, Victoria:: Liver Transplantation and Hepatology Unit, Hospital Universitari i Politécnic La Fe, Valencia, Spain
LLop, Elba:: Liver Unit, Hospital U, Puerta de Hierro, Universidad Autònoma de Madrid, CIBERehd, Madrid, Spain
Turco, Laura:: Department of Gastroenterology and Hepatology, University of Modena & Reggio Emilia and Azienda Ospedaliero-Universitaria di Modena, Modena, Italy
Indulti, Federica:: Department of Gastroenterology and Hepatology, University of Modena & Reggio Emilia and Azienda Ospedaliero-Universitaria di Modena, Modena, Italy
Gioia, Stefania:: Department of Gastroenterology and Hepatology, Centre for the Diagnosis and Treatment of Portal Hypertension, "Sapienza" University of Rome, Rome, Italy
Tosetti, Giulia:: Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Division of Gastroenterology and Hepatology, CRC "A.M.and A.Miglaivacca" Center for Liver Disease, Milan, Italy
Bitto, Niccolo:: Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Division of Gastroenterology and Hepatology, CRC "A.M.and A.Miglaivacca" Center for Liver Disease, Milan, Italy
Becchetti, Chiara:: Hepatology Group, Swiss Liver Center, University Clinic for Visceral Surgery and Medicine, Inselspital, University of Bern, Bern, Switzerland
Alvarado, Edilmar:: Liver Unit, Department of Gastroenterology Hospital Sant Pau, Barcelona, Autonomous University, Barcelona, Spain

Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Barcelona, Spain
Roig, Cristina:: Liver Unit, Department of Gastroenterology Hospital Sant Pau, Barcelona, Autonomous University, Barcelona, Spain

Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Barcelona, Spain
Diaz, Raquel:: Department of Gastroenterology and Hepatology, University Gregorio Marañón Hospital, liSGM, CIBERehd, Barcelona, Spain
Praktiknjo, Michael:: Department of Internal Medicine I, University Hospital Bonn, Bonn, Germany
Konicek, Anna-Lena:: Department of Internal Medicine I, University Hospital Bonn, Bonn, Germany
Masnou, Helena:: Liver Unit, University Hospital Germans Trias i Pujol, Badalona, Spain
Ardèvol A:: Liver Unit, University Hospital Germans Trias i Pujol, Badalona, Spain
Alvarez-Navascues, Carmen:: Liver Unit, Department of Gastroenterology and Hepatology, Hospital Universitario Central de Asturias, University of Oviedo, Oviedo, Spain
Romero, Marta:: Liver Unit, Department of Gastroenterology, Hospital Virgen de la Salud, Toledo, Spain
Scheiner, Bernhard:: Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria
Semmler, Georg:: Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria
Mandorfer, Mattias:: Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria
Damião F:: Department of Gastroenterology and Hepatology, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal
Simon-Talero, Macarena:: Liver Unit, Department of Internal Medicine, Hospital Universitari Vall d'Hebrón, Vall d'Hebron Research Institute (VHIR), Vall d'Hebron Barcelona Hospital Campus, CIBERehd, Universitat Autònoma de Barcelona, Barcelona, Spain
Gonzalez-Alayon, Carlos:: Liver Unit, Department of Gastroenterology and Hepatology, Hospital Universitario de Canarias. Tenerife, Spain
Diaz, Alba:: Department of Histopathology, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain
Garcia-Criado, Angeles:: Department of Radiology, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain
de Gottardi, Andrea:: Servizio di Gastroenterología e Epatologia, Ente Ospedaliero Cantonale, Università della Svizzera Italiana, Lugano, Switzerland
Reverter, Enric:: Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Barcelona, Spain

Liver ICU, Liver Unit, Hospital Clínic, University of Barcelona, Barcelona, Spain

Institut d'Investigacions Biomèdiques August-Pi-Sunyer (IDIBAPS), Barcelona, Spain
Blasi, Annabel:: Anesthesiology Department, Hospital Clinic-IDIBAPS, Barcelona, Spain
Genesca, Joan:: Liver Unit, Department of Internal Medicine, Hospital Universitari Vall d'Hebrón, Vall d'Hebron Research Institute (VHIR), Vall d'Hebron Barcelona Hospital Campus, CIBERehd, Universitat Autònoma de Barcelona, Barcelona, Spain
Roux, Olivier:: Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, DHU Unity, Pôle des Maladies de l'Appareil Digestif, Hôpital Beaujon, AP-HP, Clichy, France
Francoz, Claire:: Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, DHU Unity, Pôle des Maladies de l'Appareil Digestif, Hôpital Beaujon, AP-HP, Clichy, France
Noronha Ferreira C:: Department of Gastroenterology and Hepatology, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal
Reiberger, Thomas:: Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria
Rodriguez, Manuel:: Liver Unit, Department of Gastroenterology and Hepatology, Hospital Universitario Central de Asturias, University of Oviedo, Oviedo, Spain
Morillas, Rosa Maria:: Liver Unit, University Hospital Germans Trias i Pujol, Badalona, Spain
Trebicka, Jonel:: Translational Hepatology, Department of Internal Medicine I, Goethe University Frankfurt, Frankfurt, Germany

European Foundation for Study of Chronic Liver Failure, Barcelona, Spain
Bañares R:: Department of Gastroenterology and Hepatology, University Gregorio Marañón Hospital, liSGM, CIBERehd, Barcelona, Spain
Villanueva, Candid:: Liver Unit, Department of Gastroenterology Hospital Sant Pau, Barcelona, Autonomous University, Barcelona, Spain

Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Barcelona, Spain
Berzigotti, Annalisa:: Hepatology Group, Swiss Liver Center, University Clinic for Visceral Surgery and Medicine, Inselspital, University of Bern, Bern, Switzerland
Primignani, Massimo:: Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Division of Gastroenterology and Hepatology, CRC "A.M.and A.Miglaivacca" Center for Liver Disease, Milan, Italy
La Mura, Vincenzo:: Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Division of Gastroenterology and Hepatology, CRC "A.M.and A.Miglaivacca" Center for Liver Disease, Milan, Italy
Riggio, Oliviero:: Department of Gastroenterology and Hepatology, Centre for the Diagnosis and Treatment of Portal Hypertension, "Sapienza" University of Rome, Rome, Italy
Schepis, Filippo:: Department of Gastroenterology and Hepatology, University of Modena & Reggio Emilia and Azienda Ospedaliero-Universitaria di Modena, Modena, Italy
Procopet, Bogdan:: Regional Institute of Gastroenterology and Hepatology "Octavian Fodor," Hepatology Department and "Iuliu Hatieganu" University of Medicine and Pharmacy, 3rd Medical Clinic, Cluj-Napoca, Romania
Verhelst, Xavier:: Department of Gastroenterology and Hepatology, Ghent University Hospital, Ghent, Belgium
Calleja, Jose Luis:: Liver Unit, Hospital U, Puerta de Hierro, Universidad Autònoma de Madrid, CIBERehd, Madrid, Spain
Bureau, Christophe:: Department of Hepatology, Rangueil Hospital, CHU Toulouse, University Paul Sabatier of Toulouse, France
Albillos, Agustin:: Department of Gastroenterology and Hepatology, Hospital Universitario Ramón y Cajal, IRYCIS, CIBERehd, Universidad de Alcalá, Madrid, Spain
Nevens, Frederik:: Department of Gastroenterology and Hepatology, University Hospital KU Leuven, Leuven, Belgium
Tripathi, Dhiraj:: The Liver Unit, University Hospital Birmingham NHS Foundation Trust, Birmingham, United Kingdom
Rautou, Pierre-Emmanuel:: Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, DHU Unity, Pôle des Maladies de l'Appareil Digestif, Hôpital Beaujon, AP-HP, Clichy, France
Durand, Francois:: Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, DHU Unity, Pôle des Maladies de l'Appareil Digestif, Hôpital Beaujon, AP-HP, Clichy, France

Keywords

INTRAHEPATIC PORTOSYSTEMIC SHUNT; PRIMARY BILIARY-CIRRHOSIS; RENAL DYSFUNCTION; VEIN THROMBOSIS; HYPERTENSION; DISEASE; COMPLICATIONS; ASSOCIATION; GUIDELINES; REJECTION