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Challenges in Accurate Diagnosis of HCC in FALD: A Case Series
Monica Feeley1, Kali Hopkins2,3, Lauren T Grinspan1,4
1Icahn School of Medicine at Mount Sinai, New York, NY, USA.
Insights
Fontan-Associated Liver Disease (FALD) in patients with single ventricle heart disease increases hepatocellular carcinoma (HCC) risk. Diagnosing HCC in FALD is challenging due to unreliable standard imaging criteria for cirrhosis.
Area of Science:
- Cardiology
- Hepatology
- Oncology
Background:
- Single ventricle cardiac disease requires Fontan procedures for palliation.
- Fontan-Associated Liver Disease (FALD) is an inevitable complication post-Fontan procedure.
- FALD significantly elevates the risk of developing hepatocellular carcinoma (HCC).
Purpose of the Study:
- To highlight the diagnostic challenges of hepatocellular carcinoma (HCC) in patients with Fontan-Associated Liver Disease (FALD).
- To present a case series illustrating the difficulties in diagnosing HCC within this specific patient cohort.
Main Methods:
- Retrospective review of 6 cases at a single center.
- Analysis of imaging findings and diagnostic criteria used for HCC in FALD patients.
- Comparison of standard cirrhosis imaging criteria against FALD presentation.
Main Results:
- Standard imaging criteria for cirrhosis are unreliable in FALD due to heterogeneous liver parenchyma.
- Diagnosing HCC in the context of FALD presents unique challenges not addressed by current guidelines.
- The presented cases underscore the need for specialized diagnostic approaches.
Conclusions:
- Accurate diagnosis of HCC in FALD patients is complex and requires tailored imaging interpretation.
- Further research is needed to develop reliable diagnostic tools for HCC in the Fontan population.
- Early and accurate HCC detection is critical for improving outcomes in FALD patients.
Abstract:
Patients with single ventricle cardiac disease palliated with Fontan procedures inevitably develop Fontan-Associated Liver Disease (FALD), which poses a significant risk for hepatocellular carcinoma (HCC). Standard imaging criteria for the diagnosis of cirrhosis are not reliable due to parenchymal heterogeneity of FALD. We present 6 cases to demonstrate our center's experience and the challenges in diagnosing HCC within this patient population.

