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Evaluating the Longevity of the Fontan Pathway
John M Kelly1,2,3, Gabriel J M Mirhaidari4,5, Yu-Chun Chang4,5
1Center for Regenerative Medicine, Abigail Wexner Research Institute At Nationwide Children's Hospital, Columbus, OH, USA. john.kelly@nationwidechildrens.org.
Insights
Children undergoing Fontan palliation experience long-term complications. This review examines how graft materials impact Fontan pathway changes and patient outcomes, aiding better follow-up for single ventricle patients.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Surgical Innovation
Background:
- Single ventricle physiology presents significant challenges for pediatric patients.
- Fontan palliation is a complex procedure with diverse long-term complications.
- Variability in surgical techniques and graft materials complicates patient evaluation.
Purpose of the Study:
- To review changes within the Fontan pathway related to graft materials.
- To establish the relationship between graft material, pathway changes, and patient outcomes.
- To provide a comprehensive overview for evaluating single ventricle patients.
Main Methods:
- Historical review of the Fontan procedure's evolution.
- Analysis of design changes and biomaterial incorporation.
- Delineation of Fontan pathway changes: stenosis, differential growth, thrombosis, calcification.
Main Results:
- Graft material significantly influences the development of Fontan pathway complications.
- Specific pathway changes correlate with altered hemodynamics and functional capacity.
- Understanding these changes is crucial for managing Fontan-associated comorbidities.
Conclusions:
- Graft material is a critical factor in Fontan pathway integrity and patient prognosis.
- Standardized diagnostic modalities are needed for consistent patient follow-up.
- Assessing pathway changes relative to hemodynamics and comorbidities improves patient care.
Abstract:
Children born with single ventricle physiology who undergo Fontan palliation face a diverse set of long-term complications. However, patient follow-up has in large part been limited to single institutional experiences without uniform application of diagnostic modalities to screen for relevant outcomes. Additionally, the use of different graft materials and variable surgical technique as part of the Fontan procedure has further complicated the evaluation of single ventricle patients. The purpose of this review is to define the changes in the Fontan pathway specific to the graft material used and its relationship to patient outcomes. As a means of introduction, we briefly review the historical evolution of the Fontan procedure with a focus on the intent behind design changes and incorporation of different biomaterials. We further delineate changes to the Fontan pathway which include the development of stenosis, differential growth, thrombosis, and calcification. Ultimately, the recognition of the changes noted within the Fontan pathway need to be assessed relative to their impact on patient hemodynamics, functional capacity, and Fontan-associated comorbidities.

