Early-Onset Gastric Varices After Fontan Completion Associated with a Pre-Existing Gastro-Renal Shunt

Gastroesophageal varices are recognized complications of Fontan-associated liver disease, but clinically significant varices have generally been reported more than 10 years after Fontan completion. The mechanisms of early variceal formation remain unclear. We report a child with tricuspid atresia who developed isolated gastric varices approximately 7 years after extracardiac Fontan completion in the presence of a pre-existing gastro-renal shunt (GRS). The patient underwent bidirectional Glenn palliation at 9 months of age. Contrast-enhanced computed tomography (CT) performed before Fontan completion incidentally demonstrated a GRS, which progressively dilated after Fontan completion. At 8 years of age, contrast-enhanced CT obtained during evaluation for hypoxemia incidentally revealed isolated gastric varices. The patient had no ascites, marked splenomegaly, thrombocytopenia, or evidence of advanced hepatic dysfunction. Cardiac catheterization showed an inferior vena cava (IVC) pressure of 14 mmHg. Given the potential risk of massive hemorrhage, the gastric varices were successfully treated with transvenous coil embolization and balloon-occluded retrograde transvenous obliteration through the GRS. No recurrence was observed during follow-up. This case suggests that a pre-existing GRS may contribute to the relatively early development of gastric varices after Fontan completion, even in the absence of markedly elevated systemic venous pressure or other clinical features of advanced portal hypertension. Because a pre-existing GRS could predispose patients to gastric variceal formation after Fontan completion, preoperative assessment of the abdominal venous anatomy using contrast-enhanced CT could be important.