Diagnostic and Therapeutic Yield of Routine Esophagogastroduodenoscopy in Patients with Fontan Palliation

Fontan-associated liver disease (FALD) is an impactful complication for adults with Fontan circulation. The presence of esophageal varices is associated with increased morbidity and mortality in patients with underlying cirrhosis. The diagnostic yield of routine esophagogastroduodenoscopy (EGD) screening in Fontan patients with clinical or radiographic evidence of cirrhosis is not well known with wide practice variation across centers. We performed a retrospective chart review of all patients with Fontan circulation who underwent an EGD from 2020 to 2023 at our center. During the study period, a total of 66 patients (51.5% male) with Fontan circulation underwent a screening EGD at a median age 30.2 years (range 19.8-60.4 years). Esophageal varices were present in 41 (62.1%) patients, of which 35 (53.0%) had grade I esophageal varices, and 6 (9.1%) had grade II. The study showed a high incidence of esophageal varices in our patients with a Fontan circulation, suggesting a role for routine EGD as a screening tool in this population. This diagnostic procedure was well tolerated with no associated complications.