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Characteristics and Survival Outcomes of Hepatocellular Carcinoma After the Fontan Operation
Benjamin E Rosenthal1, Maarouf A Hoteit2, Gentian Lluri3
1Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Insights
Early detection of hepatocellular carcinoma (HCC) in Fontan-associated liver disease (FALD) patients after the Fontan operation may improve survival. Screening is crucial for identifying early-stage liver cancer in this population.
Area of Science:
- Cardiology
- Hepatology
- Oncology
Background:
- The Fontan operation is a palliative surgery for single ventricle congenital heart disease.
- Hepatocellular carcinoma (HCC) is a rare but serious complication of Fontan-associated liver disease (FALD).
Purpose of the Study:
- To characterize HCC in patients with Fontan circulation.
- To describe tumor features, treatment strategies, and survival outcomes in this cohort.
Main Methods:
- A multicenter retrospective case-control study (2005-2021) involving adults with Fontan circulation.
- HCC cases confirmed by histology or imaging; controls matched 3:1.
- Descriptive statistics and Kaplan-Meier survival analysis were employed.
Main Results:
- 58 HCC cases diagnosed at a median age of 31 years; 68% diagnosed at early stages.
- HCC cases showed higher prevalence of advanced FALD complications compared to controls.
- Curative treatment was administered to 41%; 1-year survival was 78.9%, highest in early-stage disease.
Conclusions:
- Early-stage diagnosis of FALD-HCC is associated with improved survival.
- Highlights the critical need for HCC screening in post-Fontan operation individuals.
- Findings inform clinical management and surveillance strategies for FALD-HCC.
Background:
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).
Objectives:
The authors aim to examine characteristics of individuals with Fontan circulation diagnosed with HCC and to describe tumor characteristics, treatment, and survival outcomes of these patients.
Methods:
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.
Results:
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).
Conclusions:
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.
