Portopulmonary hypertension (PoPH) is a serious but frequently underdiagnosed complication of cirrhosis and portal hypertension. Traditionally, attention has focused on advanced PoPH, while mild or “borderline” pulmonary hemodynamic abnormalities were often considered clinically insignificant.
Portopulmonary hypertension (PoPH) is a serious but frequently underdiagnosed complication of cirrhosis and portal hypertension. Traditionally, attention has focused on advanced PoPH, while mild or “borderline” pulmonary hemodynamic abnormalities were often considered clinically insignificant. This multicenter PORTO-DETECT cohort study challenges that assumption by evaluating the prognostic impact of early PoPH, as newly defined by the 2022 ESC/ERS pulmonary hypertension criteria.
In this longitudinal study, adults with cirrhosis and portal hypertension underwent systematic right-heart catheterisation and were classified according to pulmonary hemodynamics. Importantly, the revised ESC/ERS definition recognises an early stage of pulmonary arterial hypertension characterised by only mildly elevated mean pulmonary artery pressure and pulmonary vascular resistance. Patients were followed longitudinally to assess survival, with careful adjustment for liver disease severity and transplantation as a competing event.
The key finding is that early PoPH carries a substantial and independent mortality risk, comparable in magnitude to classic, overt PoPH. Patients with early PoPH had markedly worse long-term survival than those with normal pulmonary pressures, despite having relatively subtle hemodynamic abnormalities and similar degrees of liver dysfunction. Even mild elevations in pulmonary vascular resistance were independently associated with poor outcomes. In contrast, post-capillary pulmonary hypertension and other unclassified profiles did not confer excess mortality risk.
These results have important clinical implications. They demonstrate that pulmonary vascular disease in cirrhosis begins earlier than previously appreciated and that waiting for overt PoPH may miss a critical window for intervention. The study supports systematic screening for pulmonary hypertension in patients with cirrhosis, particularly using the updated ESC/ERS criteria, and highlights the need for closer follow-up and consideration of earlier targeted management in selected patients.
In summary, early PoPH is not benign. Recognizing and monitoring this condition may meaningfully improve risk stratification and outcomes in patients with cirrhosis.
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