This retrospective-prospective cohort study investigates portal sinusoidal vascular disorder (PSVD), a liver vascular disease definitively diagnosed via liver biopsy. Addressing PSVD's poorly defined natural history, unclear prognostic determinants and the scarcity of large-scale systematic research in China, the study enrolls histopathologically confirmed patients, collects baseline clinicopathological, laboratory and imaging data, tracks endpoints including transplant-free survival, liver-related events and portal vein thrombosis changes, and conducts prognostic analyses using Cox regression, Kaplan-Meier method and Fine-Gray model, aiming to characterize disease features, identify key prognostic factors, verify high-risk subgroups with inferior outcomes, and support precise clinical management while filling relevant domestic research gaps.
Patients histopathologically diagnosed with porto-sinusoidal vascular disorder (PSVD) via liver needle biopsy;
Liver biopsy specimen length ≥ 10 mm (or assessed as diagnostically adequate by a pathologist), with no histological evidence of cirrhosis;
Meeting the established diagnostic criteria for PSVD.
Concomitant Budd-Chiari syndrome or other hepatic venous outflow obstruction diseases;
Concomitant congenital hepatic fibrosis, hepatic sarcoidosis, or sinusoidal obstruction syndrome;
Concomitant hepatocellular carcinoma or other hepatic malignancies;
Age < 18 years;
This study includes adult patients aged 18 years and older with histopathologically confirmed porto-sinusoidal vascular disease (PSVD) via liver biopsy and no histological evidence of cirrhosis. All subjects are inpatients and outpatients from Beijing Ditan Hospital, covering subgroups with or without portal vein thrombosis, portal hypertension, known etiologies or concurrent chronic liver diseases. Patients with Budd-Chiari syndrome, hepatic venous outflow obstruction, congenital hepatic fibrosis, sinusoidal obstruction syndrome, hepatic malignancies or severely incomplete clinical data are excluded.
Cohort
Other
Not listed
Transplant-free survival
Time from study enrollment to all-cause death or liver transplantation, whichever occurs first.
Liver-related composite events
Occurrence of variceal bleeding, refractory ascites, hepatic encephalopathy, PVT progression or hepatic decompensation.
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