Portosystemic Shunt-associated Pulmonary Hypertension Multi-center Prospective Cohort Study
For patients and families
In plain language
An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- This is an observational study: the protocol does not assign a study treatment.
- Who it may be relevant to
- Registry conditions: Portosystemic Shunt, Pulmonary Hypertension. Basic parameters: from 18 years · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- Center list to be confirmed — check the primary protocol.
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
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Overview
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.
Detailed description
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.
Primary outcome measures
- Clinical worsening [Time frame: Up to 24 months]
Secondary outcome measures (4)
- Change from baseline in model for End-Stage Liver Disease (MELD) score [Time frame: Baseline, Month 12, and Month 24]
- Change from baseline in Child-Pugh Score [Time frame: Baseline, Month 12, and Month 24]
- Listing for or receipt of liver transplant or lung transplant [Time frame: Up to 24 months]
- Initiation or escalation of oral pulmonary hypertension (PH)-targeted therapy [Time frame: Up to 24 months]
Eligibility criteria
Inclusion criteria
- 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).
- 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).
- Signed informed consent and willingness to strictly adhere to the follow-up schedule.
Exclusion criteria
- 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.
- Pregnancy or lactation.
- Participation in other interventional clinical trials (drug or device) within the last 3 months.
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: No
Study design
- Observational model
- Cohort
Study locations
Center list to be confirmed — check the primary protocol.
Identifiers
NCT: NCT07481877 · PROSPECT