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Semin Liver Dis
DOI: 10.1055/a-2630-0848
Review Article

Portosinusoidal Vascular Disorder: When to Suspect and How to Manage?

Sarah Shalaby
1   Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-RareLiver), Barcelona, Spain
2   Fundació de Recerca Clínic Barcelona (FRCB-IDIABPS), CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), Barcelona, Spain
,
Wagner Enrique Ramírez-Quesada
1   Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-RareLiver), Barcelona, Spain
2   Fundació de Recerca Clínic Barcelona (FRCB-IDIABPS), CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), Barcelona, Spain
,
Asunción Ojeda
1   Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-RareLiver), Barcelona, Spain
2   Fundació de Recerca Clínic Barcelona (FRCB-IDIABPS), CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), Barcelona, Spain
,
Valeria Perez-Campuzano
1   Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-RareLiver), Barcelona, Spain
,
Anna Baiges
1   Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-RareLiver), Barcelona, Spain
2   Fundació de Recerca Clínic Barcelona (FRCB-IDIABPS), CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), Barcelona, Spain
3   Departament de Medicina i Ciències de la Salut, Universitat de Barcelona, Barcelona, Spain
,
Fanny Turon
1   Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-RareLiver), Barcelona, Spain
2   Fundació de Recerca Clínic Barcelona (FRCB-IDIABPS), CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), Barcelona, Spain
,
Carla Fuster
4   Department of Pathology, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi iSunyer (IDIBAPS), CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), University of Barcelona, Barcelona, Spain
,
Alba Díaz
4   Department of Pathology, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi iSunyer (IDIBAPS), CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), University of Barcelona, Barcelona, Spain
,
Virginia Hernández-Gea
1   Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-RareLiver), Barcelona, Spain
2   Fundació de Recerca Clínic Barcelona (FRCB-IDIABPS), CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), Barcelona, Spain
3   Departament de Medicina i Ciències de la Salut, Universitat de Barcelona, Barcelona, Spain
,
Juan Carlos García-Pagán
1   Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-RareLiver), Barcelona, Spain
2   Fundació de Recerca Clínic Barcelona (FRCB-IDIABPS), CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), Barcelona, Spain
3   Departament de Medicina i Ciències de la Salut, Universitat de Barcelona, Barcelona, Spain
› Author Affiliations
Funding This work was supported by the FIS PI23/01102 and FIS 23/00997 funded by the “Instituto de Salud Carlos III” and co-funded by the European Union. Project EJP_RD (RiTA) AC23_2/00015, funded by the Instituto de Salud Carlos III (ISCIII). From “CIBEREHD” funded by the “Instituto de Salud Carlos III.” From the “Commissioner for Universities and Research from the Department of Economy and Knowledge” of the “Generalitat de Catalunya” (AGAUR SGR2021 01115). S.S. has a Rio Hortega Grant. Current contract is funded by the “Instituto de Salud Carlos III” with charges to the European funds of the Recovery, Transformation, and Resilience Plan (Plan de Recuperación, Transformación y Resiliencia), with files code CM23/00068, pursuant to the Resolution of the Instituto de Salud Carlos III, O.A., M.P. of December 2023, granting the Rio Hortega Contracts, and “Co-Financed by the European Union.”
› Further Information
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Abstract

Portosinusoidal vascular disorders (PSVD) represent a group of rare conditions characterized by abnormalities in the liver's vascular architecture, often manifesting with clinical features of portal hypertension (PH), in the absence of cirrhosis. The pathophysiology of PSVD remains unclear, but it is frequently linked to underlying immunological disorders, medications, hematological disorders, and thrombophilia. Laboratory tests typically show preserved liver function with or without slight alteration on the transaminase profile. A key diagnostic feature is the presence of clear signs of PH alongside normal or only slightly elevated liver stiffness and hepatic venous pressure gradient. Liver biopsy remains essential for confirming the diagnosis and excluding other causes of PH and cirrhosis. However, histological examination may reveal subtle or mild changes, making expert pathological analysis and high-quality specimens crucial for an accurate diagnosis. In some cases, characteristic histological findings may be identified in patients without overt PH, which could represent an early stage of the disease. The long-term prognosis for patients with PSVD is mainly influenced by severity of the underlying condition and development of PH. However, treatments that modify the disease's natural history are still lacking, and management primarily focuses on controlling complications related to PH. Further research into the pathogenesis and potential therapeutic strategies for PSVD is needed to improve patient outcomes.

Keywords

idiopathic noncirrhotic portal hypertension - noncirrhotic portal vein thrombosis - nodular regenerative hyperplasia - portal obliterative venopathy - incomplete septal fibrosis


Publication History

Article published online:
27 June 2025

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