Fontan-associated liver disease: A review

Area of Science:

• Cardiology
• Hepatology
• Pediatric Congenital Heart Disease

Background:

• The Fontan procedure enhances long-term survival in single ventricle congenital heart disease.
• Fontan circulation leads to hemodynamic changes, including elevated central venous pressure and reduced cardiac output, contributing to Fontan-associated liver disease (FALD).
• Liver fibrosis is a common consequence of the Fontan operation, with increasing incidence of cirrhosis and hepatocellular carcinoma (HCC) over time.

Purpose of the Study:

• To outline the challenges in managing Fontan-associated liver disease (FALD).
• To emphasize the need for a multi-modality approach for staging liver fibrosis in FALD.
• To highlight critical interventions for failing Fontan circulation and advanced liver disease.

Main Methods:

• Multi-modality assessment including clinical, biochemical, non-invasive scores, imaging, elastography, and histology for staging liver fibrosis.
• Hemodynamic assessment to optimize Fontan physiology in cases of hepatic congestion.
• Evaluation for interventions such as percutaneous/surgical procedures, heart transplantation, or combined heart-liver transplantation.

Main Results:

• Liver fibrosis is a universal finding post-Fontan procedure.
• The duration of Fontan circulation correlates with increased risks of liver cirrhosis and HCC.
• Advanced FALD may necessitate heart transplantation or combined heart-liver transplantation.

Conclusions:

• Staging liver fibrosis in FALD demands a comprehensive, multi-modality approach.
• Management of failing Fontan circulation with hepatic congestion requires optimized hemodynamics and potential interventions.
• Establishing registries and standardized guidelines for FALD management is essential, requiring collaboration between cardiology and hepatology specialists.