Portosystemic Shunt-associated Pulmonary Hypertension Multi-center Prospective Cohort Study

Portosystemic Shunt-associated Pulmonary Hypertension Multi-center Prospective Cohort Study

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.

Portosystemic shunt-associated pulmonary hypertension is defined as a condition of abnormal pulmonary hemodynamics resulting from congenital or acquired portosystemic shunts. This clinical entity is common and presents with highly heterogeneous hemodynamic profiles, including pulmonary arterial hypertension, post-capillary pulmonary hypertension, and high-output pulmonary hypertension. Currently, no dedicated cohorts exist for this specific population, and targeted clinical data are lacking. Even for portopulmonary hypertension (PoPH), a more extensively studied subtype, previous studies in East Asian populations have primarily relied on small, single-center retrospective cohorts. Therefore, 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, thereby providing an evidence-based foundation for developing tailored diagnostic and therapeutic strategies for this population.

Inclusion Criteria:

1. Age ≥ 18 years.

2. Diagnosis of Portosystemic Shunts:Imaging evidence suggestive of portosystemic shunts (congenital or acquired) or unequivocal clinical signs of portal hypertension (e.g., splenomegaly, varices).

3. Diagnosis of Pulmonary Hypertension (PH):

   • 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).

4. Signed informed consent and willingness to strictly adhere to the follow-up schedule.

Exclusion Criteria:

1. PH caused by other reasons
2. Hepatocellular carcinoma (HCC) exceeding the Milan criteria.
3. Active extrahepatic malignancy.
4. Transjugular intrahepatic portosystemic shunt (TIPS) placement within the previous month.
5. Pregnancy or lactation.
6. Participation in other interventional clinical trials (drug or device) within the last 3 months.