Porto-sinusoidal vascular liver disorder with portal hypertension: Natural History and Long-Term Outcome.

Autores de IIS La Fe

• Victoria Aguilera Sancho-Tello

  Autor

• Mercedes Rodríguez Serna

  Autor

Participantes ajenos a IIS La Fe

• Magaz, Marta
• Giudicelli-Lett, Heloise
• G Abraldes, Juan
• Nicoara-Farcau, Oana
• Turon, Fanny
• Rajoriya, Neil
• Goel, Ashish
• Raymenants, Karlien
• Hillaire, Sophie
• Tellez, Luis
• Elkrief, Laure
• Procopet, Bogdan
• Orts, Lara
• Nery, Filipe
• Shukla, Akash
• Larrue, Helene
• 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
• Olivas, Pol
• Fortea, Jose Ignacio
• Masnou, Helena
• Puente, Angela
• Ardevol, Alba
• Navascues, Carmen A
• Romero-Gutierrez, Marta
• Scheiner, Bernhard
• Semmler, Georg
• Mandorfer, Mattias
• Damiao, Filipe
• Baiges, Anna
• Ojeda, Asuncion
• Simon-Talero, Macarena
• Gonzalez-Alayon, Carlos
• Diaz, Alba
• Garcia-Criado, Angeles
• De Gottardi, Andrea
• Hernandez-Guerra, Manuel
• Genesca, Joan
• Drilhon, Nicolas
• Ferreira, Carlos Noronha
• Reiberger, Thomas
• Morillas, Rosa Maria
• Crespo, Javier
• Trebicka, Jonel
• Banares, Rafael
• Villanueva, Candid
• Berzigotti, Annalisa
• Primignani, Massimo
• La Mura, Vincenzo
• Riggio, Oliviero
• Schepis, Filippo
• Verhelst, Xavier
• Calleja, Jose Luis
• Bureau, Christophe
• Albillos, Agustin
• Nevens, Frederik
• Hernandez-Gea, Virginia
• Tripathi, Dhiraj
• Rautou, Pierre-Emmanuel
• Garcia-Pagan, Juan Carlos
• ERN RARE-LIVER; a study of VALDIG, an EASL consortium

Grupos

• Dermatología y Regeneración Tisular

• Hepatología, Cirugia Hepatobiliopancreática y Trasplantes

Abstract

BACKGROUND & AIMS: Current knowledge of the natural history of patients with porto-sinusoidal vascular disorder (PSVD) is derived from small studies. The aim of the present study was to determine natural history and prognostic factors using a large multicenter cohort of PSVD patients. METHODS: Retrospective multicentric study of PSVD patients and signs of portal hypertension (PH) prospectively registered in 27 centers. RESULTS: 587 patients were included, median age of 47 years and 38% were women. Four-hundred and one patient had an associated condition, that was graded as severe in 157. Median follow-up was 68 months. At diagnosis, 64% of patients were asymptomatic while 36% had a PH-related complication: PH-related bleeding in 112 patients; ascites in 117 and hepatic encephalopathy in 11. In those not presenting with bleeding, the incidence of first bleeding was of 15% at 5 years, with a 5-year rebleeding rate of 18%. Five-year cumulative incidence of new or worsening ascites was of 18% and of developing PVT of 16%. Fifty (8.5%) patients received a liver transplantation and 109 (19%) died, including 55 non-liver related death. Transplant-free survival was 97%, and 83% at 1 and 5 years. Variables independently associated with transplant-free survival were age, ascites, serum bilirubin, albumin and creatinine levels at diagnosis and severe associated conditions. This allowed the creation of a Nomogram that accurately predicted prognosis. CONCLUSIONS: Prognosis of PSVD is strongly determined by the severity of the associated underlying conditions and parameters of liver and renal function.

Copyright © 2024 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

Keywords

• Porto-sinusoidal vascular disorder; idiopathic portal hypertension; natural history