Abdominal Imaging Surveillance in Adult Patients After Fontan Procedure: Risk of Chronic Liver Disease and Hepatocellular Carcinoma

Purpose To assess the prevalence of chronic liver disease (CLD) and hepatocellular carcinoma (HCC) in adult patients who had surveillance imaging after Fontan procedure. Methods Institutional review board–approved, Health Insurance Portability and Accountability Act–compliant retrospective study evaluated electronic medical records including radiology reports and clinical notes for adult patients after Fontan procedure between January 1993 and January 2016. Abdominal ultrasound, computed tomography, and magnetic resonance imaging reports were reviewed for changes of CLD and HCC. Existence of concomitant viral hepatitis was also recorded. Results A total of 145 patients (male: 78 and female: 67) had surveillance imaging after Fontan procedure. In all, 78% (113/145) had ongoing imaging surveillance (median follow-up 3.05 years, IQR: 0.75-5.3 years); 19% (21/113) had an initial normal study and remained normal throughout follow-up; 19% (21/113) had an initial normal study with subsequent imaging reporting changes of CLD; and 62% (71/113) had existing changes of CLD on initial study. HCC was identified in 5 patients (median 22 years post-Fontan, IQR: 10-29 years), 4 of which had a normal initial study. Only 1 patient with HCC had concomitant viral hepatitis C infection. Conclusion Radiologists should be aware that CLD is exceedingly common in post-Fontan cardiac physiology, and surveillance imaging is warranted given the risk of HCC.