Early PoPH linked to mortality in patients with cirrhosis

The use of the 2022 ESC/ERC definitions of pulmonary arterial hypertension (PAH) has led to the identification of cirrhosis patients with early-stage portopulmonary hypertension (PoPH), who are at greater risk of death, reports a study.

PoPH, a severe complication of cirrhosis and portal hypertension, is characterized by mild pulmonary vascular resistance elevation.

Overall, 428 adults with cirrhosis and portal hypertension who underwent right-heart catheterization between 2015 and 2023 were included in this longitudinal, multicentre, observational cohort study. Participants were stratified into normal, early PoPH, classic PoPH, post-capillary pulmonary hypertension, and unclassified profiles.

The investigators analysed all-cause mortality using multivariable Cox regression and competing-risk models, with liver transplantation as an intercurrent event.

Three-year survival rates over a median follow-up duration of 20 months were 76.7 percent in the normal mean pulmonary arterial pressure (mPAP), 49.5 percent in the early PoPH, and 42.0 percent in the classic PoPH groups.

After adjusting for age, sex, liver function, and portal hypertension severity, both early PoPH (hazard ratio [HR], 3.5, 95 percent confidence interval [CI], 1.9‒6.3; p<0.01) and classic PoPH (HR, 4.5, 95 percent CI, 2.6‒7.6; p<0.01) remained independently associated with a higher risk of mortality compared with normal mPAP.

These associations persisted in competing-risk analysis. On the other hand, post-capillary pulmonary hypertension and unclassified groups showed no significant difference from the normal mPAP cohort.

“Applying the 2022 ESC/ERC definitions of PAH identifies a subset of patients with cirrhosis with early-stage PoPH … who nevertheless face a markedly increased risk of death, emphasizing the need for systematic screening and early targeted intervention,” the investigators said.