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Updated: Sep 15, 2025

Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
The Liver in Heart Failure and Adults with Congenital Heart Disease
Lorena Millo1, Matthew Kappus2
1Department of Internal Medicine, Duke University Medical Center, Durham, NC, USA.
Insights
Heart failure commonly causes liver disease, especially in congenital heart disease (CHD) patients. Fontan-associated liver disease (FALD) in single-ventricle CHD requires multidisciplinary management for better patient outcomes.
Area of Science:
- Cardiology
- Hepatology
- Pediatric Congenital Heart Disease
Background:
- Liver disease frequently complicates heart failure due to hepatic congestion and reduced cardiac output.
- Congenital heart disease (CHD) patients face heightened risks of hepatic injury due to complex physiology and surgical interventions.
- Single-ventricle CHD patients undergoing the Fontan procedure are particularly susceptible to Fontan-associated liver disease (FALD).
Purpose of the Study:
- To highlight the challenges in screening and managing Fontan-associated liver disease (FALD).
- To emphasize the need for a multidisciplinary approach in addressing liver disease within the context of CHD.
- To deepen the understanding of the pathophysiology of liver disease in CHD patients.
Main Methods:
- Review of existing literature on liver disease in heart failure and CHD.
- Analysis of physiological factors contributing to hepatic injury in Fontan patients.
- Discussion of current screening and management strategies for FALD.
Main Results:
- FALD is a significant complication in single-ventricle CHD patients post-Fontan procedure.
- Hepatic congestion and impaired perfusion are key mechanisms linking CHD to liver injury.
- Current management of FALD presents considerable challenges.
Conclusions:
- A coordinated, multidisciplinary approach is essential for effective FALD screening and management.
- Further research is needed to improve understanding and treatment of liver disease in CHD.
- Early recognition and intervention are crucial for improving long-term outcomes in FALD patients.
Abstract:
Liver disease is common in patients with heart failure, often resulting from hepatic congestion and decreased perfusion due to reduced cardiac output. Patients with congenital heart disease (CHD) are particularly susceptible to hepatic injury due to their complex physiology, both before and following surgical interventions. Individuals with single-ventricle CHD who have undergone the Fontan procedure are especially prone to developing Fontan-associated liver disease (FALD). Screening and managing FALD remains challenging, necessitating a multidisciplinary approach to guide patient-care and to deepen the understanding of liver disease in the context of CHD.
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