This study aims to establish a multi-center registry cohort of portosystemic shunt-associated pulmonary hypertension, with the goal of clarifying the epidemiology, clinical features, phenotypic classification, response to targeted therapy, and prognostic outcomes in patients with portosystemic shunt-associated pulmonary hypertension.
Age ≥ 18 years.
Diagnosis of Portosystemic Shunts:Imaging evidence suggestive of portosystemic shunts (congenital or acquired) or unequivocal clinical signs of portal hypertension (e.g., splenomegaly, varices).
Confirmed by Right Heart Catheterization (RHC): mPAP > 20 mmHg; OR
Highly suspected by Echocardiography: Peak TRV > 3.4 m/s or compliant with ESC/ERS guidelines for high probability of PH (Note: RHC is encouraged for all enrolled patients).
PH caused by other reasons
Hepatocellular carcinoma (HCC) exceeding the Milan criteria.
Active extrahepatic malignancy.
Transjugular intrahepatic portosystemic shunt (TIPS) placement within the previous month.