Fontan-associated liver disease.

Area of Science:

• Cardiology
• Hepatology
• Radiology

Background:

• Fontan surgery corrects complex congenital heart defects but leads to chronic venous congestion.
• This chronic congestion results in Fontan-associated liver disease (FALD), a progressive condition.

Purpose of the Study:

• To review the pathophysiology of Fontan-associated liver disease.
• To describe its histologic changes.
• To outline its radiologic manifestations.

Main Methods:

• Literature review focusing on pathophysiology, histology, and imaging findings of FALD.
• Synthesis of data from relevant studies on Fontan circulation and liver sequelae.

Main Results:

• FALD involves structural and functional liver changes due to Fontan surgery-induced hemodynamic alterations.
• Histologic changes include fibrosis and cirrhosis.
• Radiologic findings encompass altered liver size/shape, signal intensity changes, vascular abnormalities, and focal lesions.

Conclusions:

• FALD is a direct consequence of Fontan surgery's hemodynamic effects.
• Radiologic manifestations are diverse, including benign nodules and hepatocellular carcinoma.
• Increased Fontan patient numbers necessitate radiologist familiarity with FALD and its imaging surveillance.