Prediction of hepatocellular carcinoma in patients with Fontan-associated liver disease using gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid magnetic resonance imaging

Aim: The unique feature of Fontan circulation is elevated central venous pressure, which causes Fontan-associated liver disease (FALD). FALD is associated with a high incidence of hepatocellular carcinoma (HCC). Performing biopsies in patients with FALD is difficult as a result of warfarinization; gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging (MRI), a noninvasive examination, is useful for characterizing liver disease and detecting HCC. However, few studies have reported the detailed features of Gd-EOB-DTPA MRI, and the association between these findings and prognosis. Thus, this study aimed to investigate the utility of Gd-EOB-DTPA MRI to predict HCC development in patients with FALD.

Methods: This study enrolled 44 patients with FALD (mean age 25 years) who underwent Gd-EOB-DTPA MRI. The hepatobiliary phase images were scored semiqualitatively, and the patients were classified into the mild (0-1 point) or severe group (≥2 points). The endpoint was HCC, and event-free survival was analyzed using Kaplan-Meier and log-rank tests.

Results: The severe group included 19 patients. During a mean follow-up of 58 months, HCC developed in six patients. Kaplan-Meier analysis revealed that patients in the severe group had a significantly poorer prognosis than those in the mild group (p = 0.0053). The Fibrosis-4 index and liver-to-spleen ratio of patients with HCC were moderate.

Conclusions: Gd-EOB-DTPA MRI can be used to classify disease severity and predict the prognosis of patients with FALD.