CT and MR imaging findings of the livers in adults with Fontan palliation: an observational study

Purpose

To describe liver imaging findings and complications on computed tomography (CT) or magnetic resonance imaging (MRI) in adults with Fontan palliation and investigate whether imaging features show correlations with clinical and physiological parameters.

Methods

Our Institutional Review Board approved this retrospective study. Two blinded abdominal radiologists reviewed abdominal CT (n = 21) and MRI (n = 16) images between September 2011 and October 2017 in 37 adults (median age 27 years, interquartile range 21–36 years, 14 males [38%]) with a Fontan palliation (median post-Fontan duration 22 years, interquartile range 19–28 years). Correlation between CT/MRI findings and clinical parameters including laboratory results within 6 months of CT/MRI examinations was assessed by Spearman's rank correlation coefficient.

Results

Lobulated hepatic surface and blunt hepatic edge were seen in 92% (34/37) and 95% (35/37) of patients, respectively. Surface nodularity was noted in 32% (12/37). In 7 patients, there were 11 hepatic nodules which showed arterial-phase hyperenhancement and washout. Among them, 2 were biopsy-proven hepatocellular carcinomas (HCCs), and the remaining 9 were focal nodular hyperplasia (FNH)-like nodules. Suprahepatic inferior vena cava (IVC) diameter showed positive correlations with post-Fontan duration (p < 0.01), serum gamma-glutamyl transferase (p < 0.01), and total bilirubin (p < 0.01).

Conclusion

The livers in post-Fontan adults show a unique morphology of blunt edge and lobulating surface with occasional nodularity. There is a diagnostic challenge in distinguishing HCCs from FNH-like nodules in post-Fontan population due to overlapping imaging findings. Suprahepatic IVC diameter is a potentially useful imaging marker that reflects hepatic dysfunction in Fontan palliation.