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Comparative Analysis of Respiratory and Functional Outcomes in Children Post-Fontan Procedure Versus Healthy Peers
João Bruno Piantino Dias Moura Silveira1, Aida Luiza Ribeiro Turquetto2, Luciana Patrick Amato2
1Pediatric Cardiac Surgery Unit, Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (InCor-FMUSP), São Paulo, Brazil. joaobrunods@hotmail.com.
Insights
Fontan patients aged 8-12 years show reduced pulmonary function and exercise capacity compared to healthy peers. However, their diaphragm muscle characteristics and quality of life remain similar, suggesting potential for targeted interventions.
Area of Science:
- Pediatric Cardiology
- Respiratory Medicine
- Exercise Physiology
Background:
- Respiratory muscle training is known to improve functional capacity and pulmonary function in individuals with Fontan physiology.
- However, specific characteristics of the diaphragm muscle in children with Fontan circulation are not well understood.
- This knowledge gap hinders the development of comprehensive management strategies for these patients.
Purpose of the Study:
- To compare respiratory function, maximal and submaximal functional capacities, and quality of life between children with Fontan physiology and healthy controls.
- To assess diaphragm muscle mobility and thickness in Fontan patients compared to a healthy control group.
- To elucidate the diaphragm's role in the functional limitations observed in pediatric Fontan patients.
Main Methods:
- A cross-sectional study involving 27 Fontan patients (FG) and 18 healthy controls (CG), aged 8-12 years.
- Evaluations included spirometry for pulmonary function, manovacuometry for respiratory muscle strength, chest ultrasound for diaphragm characteristics, cardiopulmonary exercise testing and 6-minute walk test (6MWT) for functional capacity, and the AUQEI questionnaire for quality of life.
- Descriptive analysis and intergroup comparisons were conducted.
Main Results:
- Fontan patients exhibited significantly impaired pulmonary function (e.g., lower FEV1 and FVC) and reduced functional capacity (e.g., lower peak VO2 and 6MWT distance) compared to controls.
- Respiratory muscle strength was also significantly lower in the Fontan group.
- Interestingly, diaphragm thickness, mobility, and quality of life were found to be similar between the Fontan and control groups.
Conclusions:
- Children with Fontan physiology demonstrate reduced pulmonary function, exercise capacity, and respiratory muscle strength compared to healthy children.
- Despite these deficits, diaphragm muscle characteristics and quality of life appear preserved in this cohort.
- These findings suggest that while the diaphragm may not be primarily affected, other respiratory and systemic factors contribute to functional limitations in Fontan patients, highlighting the need for further research into targeted respiratory interventions.
Abstract:
Studies have shown that respiratory muscle training enhances functional capacity and pulmonary function in Fontan patients. However, diaphragm muscle characteristics in Fontan children have not been fully elucidated. The aim of this study was to compare respiratory function, maximal and submaximal functional capacities, and quality of life, as well as to assess diaphragm mobility and thickness, between Fontan patients aged 8 to 12 years and healthy individuals. This cross-sectional study included 45 children-27 Fontan patients, Fontan Group (FG) and 18 healthy control Group (CG) subjects. Different examinations were performed: spirometry was used to analyze pulmonary volume and capacity; manovacuometry was used to determine respiratory muscle strength; chest ultrasound was used to determine diaphragm muscle characteristics; cardiopulmonary exercise and the six-minute walk test (6MWT) were used to determine functional capacity; the AUQEI questionnaire was used to determine quality of life. Descriptive analysis and intergroup comparisons were performed for all the data. Compared with the CG, the FG exhibited impaired pulmonary function and reduced functional capacity. Significant differences in median values were noted for forced expiratory volume in one second (FEV1): 2.39 L/min, p = 0.002; forced vital capacity (FVC): 1.73 vs. 3.06 L/min, p = 0.002; maximal inspiratory pressure: - 73 vs. - 117 cmH2O, p = 0.007; absolute peak VO2: 1.09 vs. 1.51 L/min, p < 0.001; relative peak VO2: 31.9 vs. 42.5 mL/kg/min, p = 0.003; and 6MWT distance: 420 vs. 586 m, p < 0.001. Diaphragmatic thickness, mobility and quality of life were similar between the groups. Despite the reduced functional capacity, impaired pulmonary volume and capacity, and respiratory muscle weakness of the FG compared to those of the CG, the diaphragm characteristics and quality of life were similar between the groups.
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