Abstract: Background & Aims: Portopulmonary hypertension (PoPH) is a severe complication of cirrhosis and portal hypertension. In 2022, a European task force revised the diagnostic criteria for pulmonary arterial hypertension (PAH), defining early stages as a mean pulmonary arterial pressure (mPAP) of 20.5-24.5 mmHg and pulmonary vascular resistance (PVR) >2 Wood units. We evaluated the prognostic value of these revised criteria in patients with cirrhosis.
Methods: In this longitudinal, multicenter, observational cohort, 428 adults with cirrhosis and portal hypertension underwent right-heart catheterization between 2015 and 2023 and were stratified into five groups: normal, early PoPH, classic PoPH, post-capillary pulmonary hypertension, and unclassified profiles. All-cause mortality was analyzed by multivariable Cox regression and competing-risk models, with liver transplantation as an intercurrent event.
Results: Over a median follow-up time of 20.0 months (IQR 8.0-36.0), 3-year survival rates were 76.7%, 49.5%, and 42.0% in the normal mPAP, early PoPH, and classic PoPH groups, respectively. After adjustment for age, sex, liver function, and portal hypertension severity, both early PoPH (hazard ratio 3.5; 95% CI 1.9-6.3; p <0.01) and classic PoPH (hazard ratio 4.5; 2.6-7.6; p <0.01) remained independent predictors of mortality vs. normal mPAP; these associations persisted in competing-risk analysis, whereas post-capillary pulmonary hypertension and unclassified groups did not differ from the normal mPAP cohort.
Conclusion: Applying the 2022 ESC/ERC definitions of PAH identifies a subset of patients with cirrhosis with early-stage PoPH, characterized by mild pulmonary vascular resistance elevation, who nevertheless face a markedly increased risk of death, emphasizing the need for systematic screening and early targeted intervention.
