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Nutritional Management of Patients with Fontan Circulation: A Potential for Improved Outcomes from Birth to Adulthood
Letizia Baldini1,2, Katia Librandi1, Chiara D'Eusebio3
1Postgraduate School of Pediatrics, University of Turin, 10126 Turin, Italy.
Insights
Malnutrition is a significant concern for children with Fontan circulation (FC), impacting growth and outcomes. This review provides practical nutritional management guidelines for FC patients across all life stages.
Area of Science:
- Pediatric Cardiology
- Nutritional Science
- Congenital Heart Disease Management
Background:
- Fontan circulation (FC) is a palliative surgical procedure for univentricular heart defects.
- Malnutrition is prevalent in FC patients, leading to adverse growth, neurodevelopmental, and mortality outcomes.
- Current specific nutritional guidelines for FC patients are lacking.
Purpose of the Study:
- To analyze the role of nutritional support in Fontan circulation patients.
- To provide a practical approach to nutritional management throughout the lifespan.
- To address nutritional challenges in failing Fontan circulation.
Main Methods:
- Review of existing literature on Fontan circulation and nutrition.
- Analysis of growth faltering, malnutrition pathogenesis, detection, and evaluation.
- Summary of nutritional issues across different life stages (infancy to adulthood).
Main Results:
- Faltering growth and malnutrition are significant burdens in univentricular heart patients.
- Nutritional issues vary across developmental stages, from infancy through adulthood.
- Nutritional management is particularly challenging in patients with failing Fontan circulation.
Conclusions:
- Adequate nutritional support is crucial for improving outcomes in Fontan circulation patients.
- A comprehensive, stage-specific nutritional strategy is needed.
- Further research and guidelines are essential for optimizing nutritional care in this population.
Abstract:
Fontan circulation (FC) is a surgically achieved palliation state offered to patients affected by a wide variety of congenital heart defects (CHDs) that are grouped under the name of univentricular heart. The procedure includes three different surgical stages. Malnutrition is a matter of concern in any phase of life for these children, often leading to longer hospital stays, higher mortality rates, and a higher risk of adverse neurodevelopmental and growth outcomes. Notwithstanding the relevance of proper nutrition for this subset of patients, specific guidelines on the matter are lacking. In this review, we aim to analyze the role of an adequate form of nutritional support in patients with FC throughout the different stages of their lives, in order to provide a practical approach to appropriate nutritional management. Firstly, the burden of faltering growth in patients with univentricular heart is analyzed, focusing on the pathogenesis of malnutrition, its detection and evaluation. Secondly, we summarize the nutritional issues of each life phase of a Fontan patient from birth to adulthood. Finally, we highlight the challenges of nutritional management in patients with failing Fontan.
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