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Updated: Jun 3, 2026

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Published on: May 2, 2025
Liver disease in the patient with Fontan circulation
Fred M Wu1, Chinweike Ukomadu, Robert D Odze
1Boston Adult Congenital Heart Service, Department of Cardiology, Children's Hospital Boston, Boston, MA 02115, USA. fred.wu@cardio.chboston.org
The Fontan procedure, a surgery for single-ventricle physiology, can lead to liver issues over time. This review examines liver disease in Fontan survivors and its impact on long-term health.
Area of Science:
- Cardiology
- Hepatology
- Pediatric Surgery
Background:
- The Fontan procedure is the standard surgical approach for complex single-ventricle congenital heart disease.
- Long-term Fontan circulation can lead to various complications, including liver dysfunction.
- Liver abnormalities and clotting cascade issues are recognized in Fontan patients but their clinical significance is not fully understood.
Purpose of the Study:
- To review current data on liver disease in the Fontan population.
- To understand the clinical significance of hepatic dysfunction in Fontan survivors.
- To identify unanswered questions regarding liver health in this cohort.
Main Methods:
- Literature review of studies on Fontan procedure outcomes.
- Analysis of data on liver abnormalities and hepatic dysfunction in Fontan patients.
- Synthesis of information on the impact of liver disease on Fontan circulation.
Main Results:
- Fontan survivors show increasing prevalence of subclinical hepatic dysfunction.
- Liver disease contributes to adverse outcomes in individuals with Fontan circulation.
- Abnormalities in the clotting cascade are frequently observed.
Conclusions:
- Liver disease is an increasingly recognized complication in the aging Fontan population.
- Further research is needed to clarify the long-term consequences of hepatic dysfunction in Fontan survivors.
- Understanding and managing liver health is crucial for improving outcomes in Fontan patients.
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