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

NCT07481877 | Not Yet Recruiting

• 1 other identifier: PROSPECT
• Study Type: observational
• Target: 300
• Locations: 0 countries
• Sites: N/A

Brief Summary

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.

Conditions

Portosystemic Shunt; Pulmonary Hypertension

Keywords

Portosystemic Shunt; Pulmonary Hypertension; POPH; Portal hypertension; Cirrhosis

Outcome Measures

Primary Outcomes (1)

• Clinical worsening

  The rate of clinical worsening during the follow-up period, which is a composite endpoint comprising all-cause death, decline in exercise capacity \[defined as a ≥15% reduction in 6-minute walk distance (6MWD) compared with baseline\], deterioration in World Health Organization (WHO) functional class, and non-elective hospitalizations for pulmonary hypertension (due to worsening heart failure or initiation of parenteral prostanoids).

  Up to 24 months

Secondary Outcomes (4)

• Change from baseline in model for End-Stage Liver Disease (MELD) score

  Baseline, Month 12, and Month 24

• Change from baseline in Child-Pugh Score

  Baseline, Month 12, and Month 24

• Listing for or receipt of liver transplant or lung transplant

  Up to 24 months

• Initiation or escalation of oral pulmonary hypertension (PH)-targeted therapy

  Up to 24 months

Study Arms (1)

Portosystemic shunt-associated pulmonary hypertension

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

patients with portosystemic shunt-associated pulmonary hypertension

You may qualify if:

• 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.

You may not qualify if:

• 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.

Sponsors & Collaborators

• Chinese Pulmonary Vascular Disease Research Group: lead

MeSH Terms

Conditions

Hypertension, Pulmonary; Hypertension, Portal; Fibrosis

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases; Liver Diseases; Digestive System Diseases; Pathologic Processes; Pathological Conditions, Signs and Symptoms

Central Study Contacts

Zhihong Liu, MD, PhD

CONTACT

+86 13269276067; zhihongliufuwai@163.com

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: March 8, 2026
• First Posted: March 19, 2026
• Study Start: April 1, 2026
• Primary Completion (Estimated): April 1, 2031
• Study Completion (Estimated): April 1, 2031
• Last Updated: March 19, 2026

Record last verified: 2026-03