Flexibility: A Hidden But Trainable Morbidity in Pediatric Patients with Congenital Heart Disease.

Flexibility is important for range of motion, muscular performance, and injury prevention with exercise. Promoting exercise is important for patients with congenital and pediatric acquired heart disease (CHD), yet there are a paucity of data addressing flexibility in this population. We hypothesized that flexibility was worse in pediatric patients with CHD than the general population but could be improved with directed training. Patients at Boston Children's Hospital who participated in the pediatric Cardiac Fitness Program between 09/2016 and 11/2022 were retrospectively analyzed. Flexibility was assessed via sit-and-reach (SaR) box. Data from baseline and 60 days into the fitness program intervention were compared to age-matched population norms, and changes over time were assessed. Analyses were also stratified by sex and history of sternotomy. Patients with paired baseline and 60-day data were analyzed (n = 46, age 8-23 years old, 52% male). The mean SaR at baseline for CHD patients was 24.3 cm, significantly lower than the population norm (p = 0.002). The mean for male (n = 24, 21.2 cm) and female (n = 22, 27.2 cm) CHD patients was significantly lower than their respective population norms (p = 0.017 and p = 0.026, respectively). After the fitness intervention, flexibility in CHD patients significantly improved to normal, including patients with a history of sternotomy. Flexibility was significantly lower in CHD patients than the general population, but normalized with training. Further research is warranted to investigate associations of flexibility with other measures of fitness, cardiovascular status, and quality of life, as well as benefits gained with training.

Positive mindset and exercise capacity in school-aged children and adolescents with congenital heart disease.

Introduction: Patients with congenital heart disease (CHD) have variable degrees of peak oxygen consumption (VO2) that can be improved with supervised fitness training. The ability to exercise is affected by anatomy, hemodynamics, and motivation. Motivation is in part related to mindset, or personal attitudes and beliefs, and a more positive mindset around exercise has been associated with better outcomes. It is unknown whether variations in measured peak VO2 in patients with CHD are related to having a positive mindset. Methods: Patient's ages 8-17 years with CHD were administered quality of life and physical activity questionnaires at the time of their routine cardiopulmonary exercise test. Those with severe hemodynamic burden were excluded. Patients were grouped based on disease classification. Mindset was evaluated via validated questionnaires including a PROMIS Meaning and Purpose (MaP) survey and an Anxiety survey. Pearson correlation coefficients were calculated to estimate the magnitude of the association between percent predicted peak oxygen consumption (pppVO2) and questionnaire scores overall and within CHD subgroups. Results: Eighty-five patients participated; median age was 14.7 years, 53% were female, 66% had complex CHD, 20% had simple CHD, and 14% had single ventricle heart disease. Mean MaP scores were significantly lower in all CHD groups compared to population norms (p < 0.001). As a group, MaP scores were positively associated with the amount of reported physical activity (p = 0.017). In patients with simple CHD, MaP scores were positively associated with pppVO2 (p = 0.015). The association was even stronger for MaP:Anxiety, with worse ratios associated with lower pppVO2 (p = 0.005). Patients with complex and single ventricle CHD did not show a similar association. Conclusions: Patients with CHD, regardless of severity, had lower meaning and purpose scores than the general population, and these scores were associated with amount of reported physical activity. In the simple CHD subset, having a more positive mindset was associated with higher peak VO2 and a more negative mindset with lower peak VO2. This relationship was not seen with more significant CHD. While underlying CHD diagnoses are not modifiable, mindset and peak VO2 are, and consideration should be given to measuring both as each may be a target for intervention.

Establishing a Comprehensive Pediatric Cardiac Fitness and Rehabilitation Program for Congenital Heart Disease.

Children and adolescents with congenital heart disease often do not have the opportunity, inclination, or education to participate in safe and effective exercise. The consequences of this behavioral pattern affect not only cardiopulmonary parameters, but also psychosocial factors, especially when lack of participation in peer activities or sports leads to isolation and further sedentary behaviors. Importantly, unlike cardiac rehabilitation programs for adults with atherosclerotic disease, the goal for congenital heart disease patients was less about "rehabilitation" and more about promotion of optimal fitness. We thus developed a comprehensive "Cardiac Fitness Program" at Boston Children's Hospital to promote exercise training, enhanced self-confidence, and motivation for patients with congenital heart disease. Since much of sustained fitness relates to consistency and behavior change, we crafted a progressive, goal-oriented exercise curriculum and augmented it with a self-learning workbook of targeted positive mindset practices to develop self-efficacy, an app for motivation and data collection, and exercise videos to demonstrate mechanics and to reiterate a positive message. We now report our experience including program structure and framework, navigating insurance, curriculum development, and outcome measures. Methods employed and barriers encountered in the initial development and execution of this program are reviewed. Key take-aways and further considerations including virtual and home-based programs are discussed.

Effect of pulmonary artery angioplasty on exercise function after repair of tetralogy of Fallot.

BACKGROUND: The slope of the minute ventilation versus CO2 production relationship (VE/VCO2 slope) is an index of gas exchange efficiency during exercise. In patients with repaired tetralogy of Fallot (rTOF), it correlates negatively with exercise capacity and is one of the best predictors of peak oxygen consumption (VO2). In these patients, the magnitude of the VE/VCO2 slope is related to the severity of pulmonary blood flow maldistribution (PBFM). The purpose of this study was to determine whether, in patients with rTOF, improvements in PBFM after a successful balloon angioplasty procedure (BAP) result in improvements in peak VO2 and gas exchange during exercise. METHODS: Seventeen patients with rTOF and residual pulmonary artery stenoses referred for BAP were recruited. Exercise tests were performed and PBFM determined before and after BAP. RESULTS: Nine patients (group 1) had a successful BAP (ie, improvement of >5 percentage points in PBFM); 8 did not (group 2). Patients in group 1 had significantly greater improvements in VE/VCO2 slope, peak VO2, and peak oxygen pulse (an index of forward stroke volume at peak exercise) than did patients in group 2. A significant correlation existed between the improvement in PBFM and the decline in the VE/VCO2 slope (r = -0.70, P = .002). Changes in peak oxygen pulse accounted for 89% of the improvement in peak VO2. CONCLUSIONS: In these patients, a successful BAP resulted in improved peak VO2 and more efficient gas exchange during exercise. The improvement in peak VO2 appeared to be mediated by an increase in forward stroke volume.