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

Porto-sinusoidal vascular disorder idiopathic portal hypertension natural history

Journal

Journal of hepatology

ISSN: 1600-0641

Titre abrégé: J Hepatol

Pays: Netherlands

ID NLM: 8503886

Informations de publication

Date de publication:
22 Aug 2024

Historique:

received: 30 10 2023

revised: 24 07 2024

accepted: 30 07 2024

medline: 26 8 2024

pubmed: 26 8 2024

entrez: 24 8 2024

Statut: aheadofprint

Résumé

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. Retrospective multicentric study of PSVD patients and signs of portal hypertension (PH) prospectively registered in 27 centers. 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. Prognosis of PSVD is strongly determined by the severity of the associated underlying conditions and parameters of liver and renal function.

Sections du résumé

BACKGROUND & AIMS OBJECTIVE

METHODS METHODS

Retrospective multicentric study of PSVD patients and signs of portal hypertension (PH) prospectively registered in 27 centers.

RESULTS 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 CONCLUSIONS

Prognosis of PSVD is strongly determined by the severity of the associated underlying conditions and parameters of liver and renal function.

Identifiants

DOI: 10.1016/j.jhep.2024.07.035 PMID: 39181213

pubmed: 39181213

pii: S0168-8278(24)02481-4

doi: 10.1016/j.jhep.2024.07.035

pii:

doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

Informations de copyright

Déclaration de conflit d'intérêts

Declaration of Competing Interest JCGP advisory for GORE, Cook and grant from Mallinckrodt and Astra-Zeneca. The remaining authors declare no conflicts of interest that pertain to this work.