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Updated: Jun 6, 2026

Wireless Telemetry Device Implantation in a Fontan Ovine Model for Continuous and Long-Term Hemodynamic Monitoring
Published on: May 2, 2025
Rare problems associated with the Fontan circulation
David J Goldberg1, Kathryn Dodds, Jack Rychik
1Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, United States of America. goldbergda@email.chop.edu
Insights
The Fontan operation offers remarkable survival for complex congenital heart disease but results in "Fontan physiology." This necessitates ongoing research into rare, life-threatening complications affecting multiple organ systems.
Area of Science:
- Cardiology
- Pediatric Cardiac Surgery
- Congenital Heart Disease
Background:
- The Fontan operation is the final stage in palliative surgery for complex congenital heart disease with a univentricular heart.
- While technical outcomes have improved, Fontan physiology deviates from normal cardiovascular function, leading to reduced efficiency and increased venous pressure.
Purpose of the Study:
- To review the implications of Fontan physiology.
- To highlight the spectrum of rare but serious complications associated with this surgical approach.
- To emphasize the need for continued research and collaboration.
Main Methods:
- Review of existing literature on the Fontan operation and its outcomes.
- Analysis of the physiological consequences of Fontan palliation.
- Identification of associated complications and challenges in patient management.
Main Results:
- Fontan physiology results in diminished cardiac output and elevated systemic venous pressure.
- Patients face risks of hepatic dysfunction, coagulation disorders, protein-losing enteropathy, and plastic bronchitis.
- Despite high survival rates, complications are complex and multi-systemic.
Conclusions:
- Fontan palliation achieves significant survival but does not restore normal physiology.
- Managing rare complications requires a deep understanding of underlying mechanisms.
- Interdisciplinary and multi-institutional collaboration is crucial for optimizing long-term care for Fontan survivors.
Abstract:
The Fontan operation, originally described for the surgical management of tricuspid atresia, is now the final surgery in the strategy of staged palliation for a number of different forms of congenital cardiac disease with a functionally univentricular heart. Despite the improved technical outcomes of the Fontan operation, staged palliation does not recreate a normal physiology. Without a pumping chamber delivering blood to the lungs, the cardiovascular system is less efficient; cardiac output is generally diminished, and the systemic venous pressure is increased. As a result, patients with "Fontan physiology" may face a number of rare but potentially life-threatening complications including hepatic dysfunction, abnormalities of coagulation, protein-losing enteropathy, and plastic bronchitis. Despite the staged palliation resulting in remarkable survival, the possible complications for this group of patients are complex, involve multiple organ systems, and can be life threatening. Identifying the mechanisms associated with each of the rare complications, and developing strategies to treat them, requires the work of many people at many institutions. Continued collaboration between sub-specialists and between institutions will be required to optimise the care for this group of survivors with functionally univentricular hearts.
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