[Fontan-associated liver disease current status and transplantation consideration]

Area of Science:

• Cardiology
• Hepatology
• Pediatric Surgery

Context:

• Fontan-associated liver disease (FALD) is a significant complication post-Fontan procedure.
• Characterized by liver fibrosis and cirrhosis, FALD has a high incidence and few typical symptoms.
• Etiology is unclear, linked to elevated central venous pressure and impaired hepatic blood flow.

Purpose:

• To review the challenges in diagnosing and monitoring FALD.
• To highlight the importance of liver biopsy for staging fibrosis.
• To discuss management strategies for FALD.

Summary:

• FALD presents diagnostic challenges due to the lack of correlation between lab/imaging data and fibrosis severity.
• Liver biopsy is the gold standard for diagnosing and staging FALD.
• A biopsy 10 years post-Fontan is recommended, with vigilance for hepatocellular carcinoma.

Impact:

• Timely diagnosis and monitoring of FALD are essential for improving patient prognosis.
• Combined heart-liver transplantation offers a viable option for severe cases.
• This review underscores the need for proactive liver surveillance in Fontan patients.