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Published on: May 2, 2025
Structural and functional uncoupling of liver performance in the Fontan circulation
Indra Neil Guha1, Sumit Bokhandi, Zaheer Ahmad
1NIHR Nottingham Digestive Diseases Centre Biomedical Research Unit, University of Nottingham, United Kingdom.
Fontan circulation patients show liver fibrosis and function comparable to viral cirrhosis patients. However, fibrosis and liver function are not closely linked in Fontan patients, unlike in cirrhosis.
Area of Science:
- Cardiology
- Hepatology
- Medical Research
Background:
- Long-standing Fontan circulation is associated with structural liver abnormalities.
- The extent of liver dysfunction in these patients remains largely unknown.
- This study investigates liver fibrosis and function in Fontan patients.
Purpose of the Study:
- To assess structural liver changes using serum fibrosis markers.
- To evaluate liver function through indocyanine green (ICG) clearance.
- To compare liver status in Fontan patients with compensated viral cirrhotic patients.
Main Methods:
- 21 stable Fontan patients and 8 compensated viral cirrhotic patients were studied.
- Standard liver profiles, Enhanced Liver Fibrosis (ELF) score, and ICG clearance were assessed.
- Plasma disappearance rate (PDR) and 15-minute retention (R15) of ICG were measured.
Main Results:
- Indocyanine clearance (PDR) and retention (R15) were similar between Fontan and cirrhotic groups.
- The degree of liver fibrosis was comparable between the two patient groups.
- A significant correlation between fibrosis markers and liver function was observed in viral cirrhotics, but not in Fontan patients.
Conclusions:
- Fontan patients exhibit global hepatic function and fibrosis levels similar to those with viral cirrhosis.
- A key difference lies in the lack of close correlation between fibrosis and liver function in Fontan patients.
- This suggests distinct pathophysiological mechanisms in liver disease progression between Fontan circulation and viral cirrhosis.
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