Surveillance of Fontan-associated liver disease: current standards and a proposal from the European Society of

Giulia Perucca1, Charlotte de Lange2, Stéphanie Franchi-Abella3

  • 1Department of Pediatric Radiology, Regina Margherita Children's Hospital, Turin, Italy.

Pediatric Radiology
|October 16, 2021
PubMed

Insights

The Fontan procedure helps children with heart defects survive into adulthood but can cause complications like liver disease. This review summarizes Fontan-associated liver disease, its follow-up, and proposes a European imaging algorithm.

Area of Science:

  • Pediatric Cardiology
  • Hepatology
  • Radiology

Background:

  • The Fontan procedure is a life-saving surgery for complex congenital heart disease, enabling patients to reach adulthood.
  • Despite its success, Fontan-associated complications, including liver disease, significantly impact long-term patient health.
  • Fontan-associated liver disease (FALD) is a progressive condition requiring specialized monitoring.

Purpose of the Study:

  • To review the current understanding of Fontan-associated liver disease (FALD) pathophysiology.
  • To outline the goals and methods for patient follow-up.
  • To propose a consensus-based radiologic follow-up algorithm for FALD in Europe.

Main Methods:

  • Literature review of Fontan-associated liver disease (FALD).
  • Analysis of current follow-up strategies.
  • Development of a consensus-based imaging algorithm by the European Society of Paediatric Radiology's Abdominal Task Force.

Main Results:

  • The review synthesizes current knowledge on FALD's physiopathology.
  • It highlights the importance of structured follow-up protocols.
  • A proposed imaging algorithm for FALD surveillance in Europe is presented.

Conclusions:

  • Fontan-associated liver disease (FALD) is a critical long-term complication requiring vigilant management.
  • Standardized radiologic follow-up is essential for early detection and intervention.
  • The proposed algorithm aims to optimize FALD monitoring across European centers.