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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.
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.
Abstract:
Fontan surgery is a life-saving procedure for newborns with complex cardiac malformations, but it originates complications in different organs. The liver is also affected, with development of fibrosis and sometimes cirrhosis and hepatocellular carcinoma. There is no general agreement on how to follow-up these children for the development of liver disease. To understand the current practice on liver follow-up, we invited members of the European Society of Paediatric Radiology (ESPR) to fill out an online questionnaire. The survey comprised seven questions about when and how liver follow-up is performed on Fontan patients. While we found some agreement on the use of US as screening tool, and of MRI for nodule characterization, the discrepancies on timing and the lack of a shared protocol make it currently impossible to compare data among centers.
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