Congenital heart disease

Characteristics and Survival Outcomes of Hepatocellular Carcinoma After the Fontan Operation

JACC AdvPublished multicenter retrospective case-control study

Authors' conclusion

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.

Study context

Population
Adults with Fontan circulation between 2005 and 2021; Hepatocellular carcinoma (HCC) cases included based on histology or imaging-based diagnosiscases of HCC
Participants
58 adults
Comparison
HCC cases compared to controls
  • Casesn = 58
Outcome
Age at HCC diagnosis
Methodological detailscontrols randomly selected in a 3:1 ratio from the center in which the case was derived
  • Controls randomly selected in a 3:1 ratio from the center in which the case was derived

Main finding

As reported in the abstract

Cases

31

median · IQR 26–38 · years

Observational data: associations, not effects.

Further findings

Stage at diagnosis

Diagnosed at very early or early stage disease
68%

Advanced Fontan-associated liver disease (FALD) (varices, ascites, splenomegaly, decreased platelets)

Higher prevalence in cases compared to controls

Treatment with curative intent (combined heart-liver transplantation, resection, or ablation)

Performed
41%

Survival · 1 year

Survival at 1 year
78.9%
Highest among those diagnosed at very early or early stage

Survival by active surveillance at diagnosis

Over half undergoing active surveillance at diagnosis; nonsignificant trend toward higher survival
P = 0.088

Original publication

Characteristics and Survival Outcomes of Hepatocellular Carcinoma After the Fontan Operation. JACC Adv. Published 12 March 2025. doi:10.1016/j.jacadv.2025.101646

Summarised from the published abstract; every number is checked against it

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