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Fontan Liver Disease: Review of an Emerging Epidemic and Management Options
Elisa Bradley1,2, Benjamin Hendrickson3,4, Curt Daniels3,4
1Department of Internal Medicine, Division of Cardiovascular Medicine, Ohio State University, Columbus, OH, USA. elisa.bradley@osumc.edu.
Insights
Adults with complex congenital heart disease undergoing the Fontan procedure face late cardiac failure and liver disease. This review proposes screening, prevention, and treatment strategies for liver complications in Fontan patients.
Area of Science:
- Cardiology and Hepatology
- Congenital Heart Disease
- Single-Ventricle Physiology
Background:
- Adults with complex congenital heart disease (single-ventricle anatomy) often undergo the Fontan procedure.
- Late cardiac failure is a common complication following the Fontan procedure.
- Liver disease is an increasingly recognized complication in this patient population.
Purpose of the Study:
- To review current knowledge on liver disease in adults with Fontan physiology.
- To propose standardized screening and prevention measures for liver complications.
- To discuss controversial treatment options, including mechanical assist devices and transplantation.
Main Methods:
- Literature review of existing studies on Fontan-associated liver disease.
- Analysis of screening protocols and prevention strategies.
- Review of outcomes for heart transplantation and combined heart-liver transplantation.
Main Results:
- There is a lack of consensus on optimal liver evaluation in Fontan patients.
- Early identification and management of liver disease are crucial.
- Transplantation outcomes vary for isolated heart versus combined heart-liver procedures.
Conclusions:
- A structured approach to liver evaluation and management is needed for Fontan survivors.
- Proactive screening and prevention can mitigate liver disease progression.
- Further research is required to optimize treatment strategies for end-stage complications.
Opinion Statement:
Adults with complex congenital heart disease that resulted in a Fontan procedure frequently experience late cardiac failure. Increasingly, liver disease is recognized as an important complication of single-ventricle anatomy and Fontan physiology; however, there is no consensus regarding liver evaluation in this population. Here, we review what is known about liver disease in this unique group and propose screening and prevention measures. We also review controversial treatment areas including assist devices and transplantation, with a review of outcomes in isolated heart and combined heart-liver transplant.
