ArticleInternational journal of cardiology. Congenital heart disease2026
Imaging findings in Fontan-associated liver disease: Results of a single center study.
Article in International journal of cardiology. Congenital heart disease, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Background: Fontan-associated liver disease (FALD) is a consequence of Fontan circulation causing liver fibrosis, cirrhosis, portal hypertension, and potentially hepatocellular carcinoma (HCC), with imaging essential to evaluation. Objectives: To describe the cross-sectional imaging features of hepatic morphology and focal liver lesions (FLLs) in patients with FALD. Methods: This retrospective single-center study included patients post-Fontan procedure (10/2016-9/2022) who underwent CT or MRI and non-targeted liver biopsy for fibrosis staging. Two observers in consensus assessed CT/MRI for imaging findings of cirrhosis, portal hypertension, and FLL (>0.8 cm) detection and characterization, including size, enhancement pattern, and MRI characteristics. A composite reference standard (pathology, multidisciplinary tumor board, imaging characteristics, or FLL stability) informed FLL diagnosis. Biopsies were staged using the Congestive Hepatic Fibrosis Score (CHFS) [range, 0(no fibrosis)-4 (cirrhosis)]. Associations between variables were analyzed using logistic regression and Fisher's exact tests. Results: Results from 41 patients [26M/15F, mean age = 29.9y, MRI, n = 24/CT, n = 17] are presented. CHFS were 1(n = 4)/2(n = 19)/3(n = 14)/4(n = 4). Imaging signs of cirrhosis were common: liver surface nodularity (n = 31) and volume redistribution (caudate lobe hypertrophy, n = 35). Varices and ascites were observed in n = 18 and n = 16 patients. 62 FLL were identified in 15 patients (mean size = 1.5 ± 0.7 cm). Diagnoses included benign-appearing enhancing lesions (n = 52 lesions/10 patients), indeterminate (n = 5 lesions/4 patients), HCC (n = 4 lesions/3 patients), and sclerosing hemangioma (n = 1). All HCC cases had CHFS = 3. No association between laboratory, CHFS, and imaging findings of cirrhosis was found (p-values>0.12). Conclusions: Imaging findings of cirrhosis are discordant with fibrosis stage in FALD. Enhancing FLLs, including HCC, are common and frequently observed in noncirrhotic liver.
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