Distal Esophageal Varices in Fontan Circulation: Hepatology | May 2026

Introduction:

Adults with Fontan-type circulation are increasingly recognized to develop Fontan-associated liver disease and portal hypertension. This prospective study explored the prevalence, anatomical distribution, and hemodynamic characteristics of esophageal varices (EV), with particular emphasis on distal versus proximal varices.

Why was this study needed?

• Fontan-associated liver disease is becoming a major long-term complication in adult survivors.
• The mechanisms underlying esophageal varices in Fontan circulation remain poorly understood.
• The clinical significance of proximal versus distal varices has not been clearly defined.
• Better risk stratification is needed to optimize surveillance and management.
• The relationship between portal hypertension, hepatic fibrosis, and Fontan hemodynamics requires clarification.

Results:

• Esophageal varices were present in over one-third of adults with Fontan circulation, with distal varices being the most common subtype.
• Distal esophageal varices identified a unique high-risk phenotype, characterized by elevated Fontan pressures, portal hypertension, severe hepatic fibrosis, and a hyperdynamic circulatory state.
• Despite advanced liver disease, variceal bleeding was uncommon, suggesting that variceal location may provide greater prognostic value than bleeding risk alone.

Clinical Impact:

This study demonstrates that distal esophageal varices are not simply an endoscopic finding but a marker of advanced Fontan-associated liver disease and severe hemodynamic impairment. Their presence should prompt comprehensive evaluation for portal hypertension and hepatic fibrosis, potentially improving long-term surveillance and multidisciplinary management.

Bottom Line:

Distal esophageal varices define a distinct hemodynamic and hepatic phenotype in patients with Fontan circulation. They serve as an important marker of advanced Fontan-associated liver disease, portal hypertension, and severe fibrosis, warranting closer clinical monitoring.