Radiologic follow-up in Fontan-associated liver disease in Europe: European Society of Paediatric Radiology survey

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

Fontan surgery survivors require liver disease monitoring, but current follow-up practices lack standardization. This study highlights discrepancies in screening and surveillance protocols among pediatric radiologists.

Area of Science:

  • Cardiology
  • Hepatology
  • Pediatric Radiology

Background:

  • Fontan surgery is crucial for complex congenital heart defects but can lead to liver complications like fibrosis, cirrhosis, and hepatocellular carcinoma.
  • There is a lack of consensus on standardized protocols for monitoring liver disease progression in Fontan patients.
  • Effective surveillance is essential for early detection and management of liver disease in this vulnerable population.

Purpose of the Study:

  • To investigate current practices and identify variations in liver follow-up protocols for Fontan procedure patients.
  • To understand the approaches used by pediatric radiologists in screening for and characterizing liver disease in Fontan survivors.
  • To assess the level of agreement on imaging modalities and timing for liver surveillance.

Main Methods:

  • An online questionnaire was distributed to members of the European Society of Paediatric Radiology (ESPR).
  • The survey included seven questions focusing on the timing and methods of liver follow-up in Fontan patients.
  • Data analysis focused on identifying common practices and areas of significant discrepancy.

Main Results:

  • While ultrasound (US) is generally agreed upon for screening, and MRI for nodule characterization, significant variations exist in the timing of follow-up.
  • Discrepancies in surveillance schedules and the absence of a unified protocol were identified.
  • The current lack of a shared protocol hinders the ability to compare liver disease data across different centers.

Conclusions:

  • Current liver follow-up practices for Fontan patients are heterogeneous, with notable disagreements on surveillance timing.
  • While imaging tools like US and MRI show some consensus, a standardized protocol is urgently needed.
  • Establishing a shared protocol is critical for consistent data collection and improved patient management.