Surveillance of Fontan-associated liver disease: current standards and a proposal from the European Society of Paediatric Radiology Abdominal Task Force
Giulia Perucca1, Charlotte de Lange2, Stéphanie Franchi-Abella3
1Department of Pediatric Radiology, Regina Margherita Children's Hospital, Turin, Italy.
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.
Abstract:
Since Francis Fontan first introduced the eponymous technique, the Fontan procedure, this type of surgical palliation has allowed thousands of children affected by specific heart malformations to reach adulthood. Nevertheless, abdominal, thoracic, lymphatic and neurologic complications are the price that is paid by these patients. Our review focuses on Fontan-associated liver disease; the purpose is to summarize the current understanding of its physiopathology, the aim of follow-up and the specific radiologic follow-up performed in Europe. Finally, we as members of the Abdominal Task Force of the European Society of Paediatric Radiology propose a consensus-based imaging follow-up algorithm.
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