Abstract
The Fontan operation is a surgical procedure to palliate single ventricle congenital heart disease. Hepatocellular carcinoma (HCC) is a rare complication of Fontan-associated liver disease (FALD).
We aim to examine characteristics of individuals with Fontan circulation diagnosed with HCC and to describe tumor characteristics, treatment, and survival outcomes of these patients.
This was a multicenter retrospective case-control study of adults with Fontan circulation between 2005 and 2021. HCC cases were included based on histology or imaging-based diagnosis. Controls were randomly selected in a 3:1 ratio from the center in which the case was derived. Descriptive statistics were used to compare groups and Kaplan-Meier survival analysis was performed.
There were 58 cases of HCC diagnosed at a median age of 31 (IQR: 26-38) years. Diagnosis was made at very early or early stage disease in 68%. Compared to controls, cases had higher prevalence of advanced FALD including varices, ascites, splenomegaly, and decreased platelets. Treatment with curative intent (combined heart-liver transplantation, resection, or ablation) was performed in 41%. Survival at 1 year was 78.9% and highest among those diagnosed at very early or early stage. Over half were undergoing active surveillance at diagnosis, which showed a nonsignificant trend toward higher survival (P = 0.088).
We describe the clinical characteristics, treatment, and survival in patients with FALD-HCC. Results suggest that adults with FALD-HCC diagnosed with early stage disease may have survival benefit. Our findings underscore the importance of HCC screening for early detection in individuals after the Fontan operation.
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