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

Fecha de publicación: 22/08/2024 Fecha Ahead of Print: 22/08/2024

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

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.