Portopulmonary hypertension: an integrated approach to diagnosis, risk stratification, and management.

Abstract

Portopulmonary hypertension (PoPH) is a complex and underrecognized form of pulmonary arterial hypertension arising in the setting of portal hypertension, in which outcomes are driven by the interplay between pulmonary vascular disease and liver dysfunction. Despite advances in hemodynamic definitions and targeted therapies, PoPH remains associated with significant morbidity and mortality, largely due to delayed diagnosis, challenges in risk stratification, and the absence of disease-specific management strategies. Recent insights support a paradigm shift from a purely hemodynamic view toward an integrated model encompassing pulmonary vascular load, right ventricular adaptation and hepatic reserve. This multidimensional framework has important implications for screening, prognostic assessment and therapeutic decision-making, particularly in the context of liver transplantation, where treatment is increasingly aimed at achieving transplant eligibility. In this review, we provide a comprehensive and clinically oriented overview of PoPH, integrating current evidence on pathophysiology, diagnosis, risk stratification and management. We highlight key limitations of existing approaches, discuss emerging therapeutic strategies and propose a conceptual framework to guide clinical practice and future research in this challenging condition.