Jesús Donate, Susana del Prado, Andrés Tenes, María Jesus del Cerro, Agustín Albillos, Luis Téllez
Hospital Universitario Ramón y Cajal and Universidad de Alcalá.
Spain
Annals of Hepatology
Ann Hepatol 2026;
DOI: 10.1016/j.aohep.2026.102466
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.
Category
Class I. Pulmonary Hypertension Associated with Liver Disease
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes