Fontan-associated liver disease: the importance of multidisciplinary teamwork in its management

Area of Science:

• Cardiology
• Hepatology
• Pediatric Surgery

Background:

• The Fontan operation is a palliative surgery for single-ventricle defects, creating unique hemodynamics.
• This Fontan circulation can lead to progressive liver disease, including fibrosis and cirrhosis, typically starting a decade post-surgery.
• Potential complications include hepatocellular carcinoma, focal nodular hyperplasia, and portal hypertension.

Purpose of the Study:

• To outline the pathogenesis of Fontan-associated liver disease (FALD).
• To emphasize the necessity of a multidisciplinary approach for effective FALD management.

Main Methods:

• Review of literature on Fontan circulation and associated liver pathology.
• Synthesis of information regarding the development and clinical manifestations of FALD.
• Discussion of collaborative care strategies.

Main Results:

• Fontan-associated liver disease (FALD) is characterized by sinusoidal fibrosis and dilatation.
• Hepato-gastrointestinal complications arise in severe cases of fibrosis and cirrhosis.
• Effective management necessitates integrated care from multiple medical specialties.

Conclusions:

• Fontan-associated liver disease (FALD) is a significant long-term complication of the Fontan operation.
• A collaborative, multidisciplinary approach involving hepatologists, gastroenterologists, surgeons, and cardiologists is crucial for optimal patient outcomes.