Luis Téllez, Luis, María Toledano, María Álvarez, Elvira Garrido-Lestache, Elena Garrido, Antonio Guerrero, Jesús Donate, Lorenzo Canova, María Torres, Cristian Perna, María Jesús del Cerro, Agustín Albillos
Hospital Universitario Ramón y Cajal, IRYCIS, CIBERehd, Universidad de Alcalá.
Spain
Hepatology
Hepatology 2025;
DOI: 10.1097/HEP.0000000000001472
Abstract
Background aims: Esophageal varices (EV) are common in adults with Fontan-type circulation and may arise via distinct hemodynamic mechanisms depending on their anatomic location. We aimed to determine the prevalence, anatomical distribution and hemodynamic correlates of EV in this population.
Approach results: In this prospective cohort study, 114 consecutive adults with Fontan-type circulation underwent combined hepatic and cardiac catheterization, esophagogastroduodenoscopy, and liver biopsy. Patients were classified by endoscopic findings into those with and without EV, and the EV cohort was further stratified by location (proximal or distal). EV were present in 36.8% (95% CI, 27.9-45.8%), including 28.9% with distal EV and 17.5% with proximal EV. Over a median follow-up of 48.9 months (IQR, 27.1-85.5), only one variceal bleeding event occurred. Patients with distal EV exhibited the highest cardiac index and pulmonary, systemic and hepatic venous pressures, as well as the lowest systemic vascular resistance (all p<0.01). Severe hepatic fibrosis was more frequent in this group (87.9%) than in patients without EV (33.8%) or those with proximal EV (25.0%). Non-invasive fibrosis markers (liver stiffness, FIB-4, Forns index, FonLiver risk score) were significantly elevated in patients with distal EV (all p<0.05), despite similar biochemical profiles.
Conclusion: Distal EV are frequent and characterize a distinct hemodynamic phenotype, featuring elevated Fontan pathway pressures, severe hepatic fibrosis, portal hypertension, and a hyperdynamic circulatory state.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing
Acquired Patient Factors Associated with Pulmonary Vascular Disease
Age Focus: 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