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Published on: May 2, 2025
The failing Fontan: etiology, diagnosis and management
David J Goldberg1, Robert E Shaddy, Chitra Ravishankar
1Division of Cardiology, The Children's Hospital of Philadelphia, 34th and Civic Center Blvd Philadelphia, PA 19104, USA. goldbergda@email.chop.edu
Insights
The Fontan operation helps children with single ventricle heart defects survive but creates abnormal circulation. Ongoing research focuses on medical therapies to manage long-term Fontan complications and reduce transplant needs.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- The Fontan operation is a palliative procedure for single-ventricle congenital heart disease.
- While improving survival, it results in a non-physiological circulation with long-term risks.
- Fontan survivors face complications including ventricular dysfunction, plastic bronchitis, and protein-losing enteropathy.
Purpose of the Study:
- To review the long-term implications of Fontan physiology.
- To discuss emerging therapies and future directions for managing Fontan survivors.
- To highlight the limitations of current Fontan circulation and the need for improved treatments.
Main Methods:
- Review of existing literature on Fontan operation outcomes.
- Analysis of current and emerging medical and surgical strategies.
- Discussion of ongoing research into mechanical support and novel therapeutics.
Main Results:
- Fontan circulation, while life-saving, leads to significant long-term morbidities.
- Medical therapies are being developed to address specific Fontan-associated complications.
- Mechanical circulatory support devices are in early investigation stages.
Conclusions:
- Continued development of targeted medical therapies is crucial for improving outcomes in Fontan survivors.
- These therapies may potentially decrease the need for cardiac transplantation.
- Further research is needed for advanced mechanical solutions, but medical management remains a key focus.
Abstract:
While the Fontan operation has facilitated the survival of a generation of children born with congenital heart disease resulting in a functional single ventricle, it does not recreate a normal circulation. Over time, survivors of the Fontan operation are at risk for ventricular dysfunction, plastic bronchitis, protein-losing enteropathy and chronic Fontan failure. New techniques and therapies are emerging to address the long-term risks associated with Fontan physiology, but as the number of survivors continues to grow, the recognition of the limitations of this circulation is increasing. Novel investigations of possible mechanical devices designed to function as a subpulmonary ventricle are underway, but are still many years away from clinical use. In the meantime, continued development of medical therapeutics targeted at the specific problems of the Fontan circulation will be beneficial and might reduce the need for cardiac transplantation.
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