Distinctive hemodynamic phenotype in Fontan-type circulation patients with distal esophageal varices

BACKGROUND AND 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 AND 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. CONCLUSIONS: Distal EVs are frequent and characterize a distinct hemodynamic phenotype, featuring elevated Fontan pathway pressures, severe hepatic fibrosis, portal hypertension, and a hyperdynamic circulatory state.