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

Autores de IDIVAL

• 

  Javier Crespo García

  Autor

  javier.crespo@scsalud.es

• 

  José Ignacio Fortea Ormaechea

  Autor

  joseignacio.fortea@scsalud.es

• 

  Ángela María Puente Sánchez

  Autor

  angelam.puente@scsalud.es

Autores ajenos al IDIVAL

• Magaz M
• Giudicelli-Lett H
• G Abraldes J
• Nicoara-Farcau O
• Turon F
• Rajoriya N
• Goel A
• Raymenants K
• Hillaire S
• Téllez L
• Elkrief L
• Procopet B
• Orts L
• Nery F
• Shukla A
• Larrue H
• Degroote H
• Aguilera V
• LLop E
• Turco L
• Indulti F
• Gioia S
• Tosetti G
• Bitto N
• Becchetti C
• Alvarado E
• Roig C
• Diaz R
• Praktiknjo M
• Konicek AL
• Olivas P
• Masnou H
• Ardèvol A
• Navascués CA
• Romero-Gutiérrez M
• Scheiner B
• Semmler G
• Mandorfer M
• Damião F
• Baiges A
• Ojeda A
• Simón-Talero M
• González-Alayón C
• Díaz A
• García-Criado Á
• De Gottardi A
• Hernández-Guerra M
• Genescà J
• Drilhon N
• Ferreira CN
• Reiberger T
• Rodríguez M
• Morillas RM
• Trebicka J
• Bañares R
• Villanueva C
• Berzigotti A
• Primignani M
• La Mura V
• Riggio O
• Schepis F
• Verhelst X
• Calleja JL
• Bureau C
• Albillos A
• Nevens F
• Hernández-Gea V
• Tripathi D
• Rautou PE
• García-Pagán JC
• ERN RARE-LIVER; a study of VALDIG, an EASL consortium

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