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;