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Updated: May 30, 2026

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Published on: May 2, 2025
Extracardiac complications of the Fontan circuit
Geetika Khanna1, Sanjeev Bhalla, Rajesh Krishnamurthy
1Mallinckrodt Institute of Radiology, Washington University School of Medicine, Campus Box 8131, 510 S. Kingshighway, St. Louis, MO 63110, USA. khannag@mir.wustl.edu
Insights
The Fontan operation treats single-ventricle heart defects. As more children survive, radiologists increasingly encounter Fontan circuit complications, especially extracardiac issues in failing Fontan circuits.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Surgical Palliation
Background:
- The Fontan operation is a critical palliative surgery for univentricular cardiac physiology.
- Improved survival rates in congenital heart disease lead to more frequent long-term Fontan circuit complications.
- Radiologists play a key role in identifying both cardiac and extracardiac issues associated with the Fontan circuit.
Purpose of the Study:
- To highlight common extracardiac complications in children with Fontan circuit failure.
- To enhance radiologists' diagnostic capabilities for Fontan-associated complications.
- To guide cardiologists in managing patients with failing Fontan circuits.
Main Methods:
- Review of imaging findings in pediatric patients with Fontan circuits.
- Analysis of common extracardiac complications.
- Correlation of imaging findings with clinical outcomes in Fontan failure.
Main Results:
- Extracardiac complications are increasingly recognized in long-term Fontan survivors.
- Specific imaging patterns are associated with Fontan circuit failure.
- Early identification of these complications is crucial for patient management.
Conclusions:
- Radiologists must be aware of potential extracardiac complications in Fontan patients.
- Prompt diagnosis aids in timely intervention and improved patient outcomes.
- Multidisciplinary collaboration is essential for managing complex Fontan-associated morbidities.
Abstract:
The Fontan operation is the primary surgical procedure used in the palliation of patients with univentricular cardiac physiology. With improved survival of children with congenital heart disease, long-term complications of the Fontan circuit are being encountered more frequently. Radiologists are more likely to see both the cardiac and extracardiac complications of the Fontan circuit. Awareness of the common extracardiac complications in children with failing Fontan circuits will aid the radiologist in making the appropriate diagnosis and guide the cardiologist caring for these patients.
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