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.
patients with portosystemic shunt-associated pulmonary hypertension
Cohort
Prospective
Not listed
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).
Change from baseline in model for End-Stage Liver Disease (MELD) score
The MELD score is a reliable measure of mortality risk in patients with end-stage liver disease, calculated using serum bilirubin, serum creatinine, and the international normalized ratio (INR). The total score ranges from 6 to 40. Higher scores indicate more severe hepatic impairment and worse prognosis in patients with portosystemic shunt-associated pulmonary hypertension. The change in MELD score from baseline will be evaluated at each specified follow-up visit.
Change from baseline in Child-Pugh Score
The Child-Pugh score assesses the severity and prognosis of chronic liver disease based on five clinical measures: total bilirubin, serum albumin, prothrombin time (INR), ascites, and hepatic encephalopathy. The total score ranges from 5 to 15, where 5-6 indicates Class A (well-compensated), 7-9 indicates Class B (significant functional compromise), and 10-15 indicates Class C (decompensated). Higher scores represent worse liver function. The change in the total Child-Pugh score from baseline will be reported.
Listing for or receipt of liver transplant or lung transplant
This measure assesses the time elapsed from study enrollment to the first documented occurrence of a transplant-related event due to the progression of portosystemic shunt-associated pulmonary hypertension. A transplant-related event is strictly defined as either being officially placed on an active waiting list for a liver or lung transplant, or the actual surgical receipt of a liver or lung transplant. The time to whichever event occurs first will be recorded.
Initiation or escalation of oral pulmonary hypertension (PH)-targeted therapy
This measure assesses the time elapsed from study enrollment to the first documented initiation or escalation of oral targeted therapy for portosystemic shunt-associated pulmonary hypertension. "Initiation or escalation" is strictly defined as the sequential addition of a new class of oral PH-targeted medication. This encompasses the clinical transition from no PH therapy to oral monotherapy, from monotherapy to double combination therapy, or from double to triple combination therapy. Dose increases of pre-existing oral medications are excluded from this definition.
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