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Hepatocellular carcinoma and the Fontan circulation: Clinical presentation and outcomes
Mathias Possner1, Timothy Gordon-Walker2, Alexander C Egbe3
1Division of Cardiology, Department of Medicine, University of Washington, Seattle, Washington, USA.
Insights
Hepatocellular carcinoma (HCC) in Fontan circulation patients is a rare but fatal complication. Early detection through meticulous liver surveillance is critical for improving survival rates in these young patients.
Area of Science:
- Cardiology
- Hepatology
- Oncology
Background:
- Fontan-associated liver disease (FALD) is a universal complication in patients with Fontan circulation.
- Hepatocellular carcinoma (HCC) is a severe and often fatal manifestation of FALD.
- Understanding HCC in this population is crucial for improving patient outcomes.
Purpose of the Study:
- To describe the clinical presentation of HCC in Fontan circulation patients.
- To identify risk factors associated with HCC development in this cohort.
- To report on the outcomes and survival rates of HCC in Fontan patients.
Main Methods:
- A multicenter case series of Fontan patients diagnosed with HCC was compiled.
- The case series was augmented with published cases and case reports.
- Data collected included clinical presentation, tumor characteristics, laboratory and hemodynamic findings, treatment, and outcomes.
Main Results:
- Fifty-four Fontan patients with HCC were analyzed; mean age at diagnosis was 30 years.
- Median HCC size was 4 cm, with 51% having liver cirrhosis at diagnosis.
- 48% of patients died within a median follow-up of 10.6 months, with a 1-year survival rate of 50%.
Conclusions:
- HCC in Fontan patients occurs at a young age with a poor prognosis.
- A significant proportion of patients present with asymptomatic disease and normal alpha-fetoprotein levels.
- Meticulous liver surveillance is essential for early detection and improved survival.
Background:
Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation.
Methods:
A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described.
Results:
Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1-50) months.
Conclusions:
HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.
