Nationwide Survey Reveals High Prevalence of Non-Swimmers among Children with Congenital Heart Defects.

BACKGROUND: Physical activity is important for children with congenital heart defects (CHD), not only for somatic health, but also for neurologic, emotional, and psychosocial development. Swimming is a popular endurance sport which is in general suitable for most children with CHD. Since we have previously shown that children with CHD are less frequently physically active than their healthy peers, we hypothesized that the prevalence of non-swimmers is higher in CHD patients than in healthy children. METHODS: To obtain representative data, we performed a nationwide survey in collaboration with the German National Register of Congenital Heart Defects (NRCHD) and the Institute for Sport Sciences of the Karlsruhe Institute for Technology (KIT). The questionnaire included questions capturing the prevalence of swimming skills and the timing of swim learning and was part of the "Motorik-Modul" (MoMo) from the German Health Interview and Examination Survey for Children and Adolescents (KiGGS). A representative age-matched subset of 4569 participants of the MoMo wave two study served as a healthy control group. RESULTS: From 894 CHD-patients (mean age of 12.5 ± 3.1 years), the proportion of non-swimmers in children with CHD was significantly higher (16% versus 4.3%; p < 0.001) compared to healthy children and was dependent on CHD severity: Children with complex CHD had an almost five-fold increased risk (20.4%) of being unable to swim, whereas in children with simple CHD, the ability to swim did not differ significantly from their healthy reference group (5.6% vs. 4.3% non-swimmers (p = not significant). CONCLUSIONS: According to our results, one in five patients with complex CHD are non-swimmers, a situation that is concerning in regard of motoric development, inclusion and integration, as well as prevention of drowning accidents. Implementation of swim learning interventions for children with CHD would be a reasonable approach.

Physical Self-Concept and Physical Activity in Children with Congenital Heart Defects-Can We Point Out Differences to Healthy Children to Promote Physical Activity?

OBJECTIVE: Children with congenital heart defects (CHD) are at high risk for cardiovascular disease in addition to their congenital disease, so it is important to motivate this group of patients to live a physically active lifestyle. A potential influencing determinant of younger children's physical performance is the physical self-concept. The objective of the present study was first to evaluate the correlation between the physical self-concept (PSC) and the participation in physical activities (PA) of a representative group of children with congenital heart disease (CHD), and second to point out differences in comparison to their healthy peer group. METHODS: Using the database of PA of the S-BAHn-Study we focused on physical self-concept assessed by the German version of the Physical Self-Description Questionnaire. We compare the obtained data of children with CHD to a representative age-matched sample of 3.385 participants of the Motorik Modul Study. RESULTS: N = 1.198 complete datasets could be included in the analyses. The mean age of patients was 11.6 ± 3.1 years. For the total cohort of patients with CHD and the reference group, PA correlated significantly with a positive PSC (p < 0.001). PA was significantly reduced in all groups of patients despite the severity of their heart defect (p < 0.001). Remarkably, PSC did not differ statistically significantly in patients with simple CHD from the reference collective (p > 0.24). CONCLUSIONS: According to this representative survey, there is a clear relation between PA and PSC in the cohort of healthy children and the group of children with CHD throughout the severity of their heart defects. Although PSC did not differ in patients with simple CHD and their healthy peer group, PA was significantly reduced. This gap invites us to reflect on how we could break new ground to promote a physically active lifestyle in children with CHD regardless of the severity of their cardiac defects.

Habitual physical activity in patients born with oesophageal atresia: a multicenter cross-sectional study and comparison to a healthy reference cohort matched for gender and age.

Oesophageal atresia (EA) is associated with life-long gastrointestinal and respiratory morbidity and other associated malformations. The aim of this study is to compare physical activity (PA) levels of children and adolescents with and without EA. A validated questionnaire (MoMo-PAQ) was used to evaluate PA in EA patients EA (4-17 years), who were randomly matched for gender and age (1:5) with a representative sample of the Motorik-Modul Longitudinal Study (n = 6233). Sports activity per week (sports index) and minutes of moderate to vigorous physical activity per week (MVPA minutes) were calculated. Correlations between PA and medical factors were analysed. In total, 104 patients and 520 controls were included. Children with EA were significantly less active at higher intensities (mean MPVA minutes 462; 95% confidence interval (CI): 370-554) compared to controls (626; 95% CI: 576-676), although there was no statistically significant difference in the sports index (187; 95% CI: 156-220 versus 220; 95% CI: 203-237). A lower mean weight-for-age and height-for-age, additional urogenital (r = - 0.20, p = 0.04) or anorectal malformation (r = - 0.24, p = 0.01) were associated with fewer MVPA minutes. For other medical factors (prematurity, type of repair, congenital heart disease, skeletal malformation or symptom load), no statistically significant association with PA was found.  Conclusion: EA patients participated in PA at a similar level but lower intensities compared to the reference cohort. PA in EA patients was largely independent of medical factors. TRIAL REGISTRATION: German Clinical Trials Register (ID: DRKS00025276) on September 6, 2021. WHAT IS KNOWN: • Oesophageal atresia is associated with a low body weight and height, delayed development of motor skills and impaired lung function and exercise capacity. WHAT IS NEW: • Patients with oesophageal atresia have a similar amount of sports activity per week but engage significantly less in moderate to vigorous physical activities compared to peers. • Physical activity was  associated with weight-for-age and height-for-age, but largely independent of symptom load and other medical factors.

[Opportunities in Differentiated School Sports Exemption - Results of An Online-Survey and Review of the Literature]. / Differenzierte Sportbefreiung als Chance: Ergebnisse eines Online-Survey und Übersicht über die aktuelle Literatur.

BACKGROUND: Physical education and sports participation in school play a major role in overall activity of children and adolescents. A differentiated school sports exemption (DSE) allows sports participation adapted to the individual's ability. METHOD: Online-survey among physicians (2019) and physical education (PE) teachers (in two waves, 2017 and 2019), on their opinion towards the concept of DSE with respect to professional and demographic parameters. Answers were scaled on a 6-point Likert scale. Means and 95% confidence intervals (95%-CI) were calculated. RESULTS: Complete questionnaires of 97 physicians and 280 teachers were included. A mean of 10,3% (95%-CI: 9,5-11,1%) of students, did not participate in PE class. In most cases, students were excused directly by their parents without consulting a physician. Most frequent reasons for exemption were missing sports gear (62%), common colds (54%), or acute injuries (50%). Chronic disease or disability was rare (8%). Most teachers did not feel well prepared to deal with children with chronic diseases or disabilities in PE class. Many physicians (24%) had never considered DSE. However, the concept was well accepted among both physicians and teachers. It was considered more practicable than exemption from being marked. DISCUSSION: According to participants, DSE is expedient and feasible. As a secondary option, students with chronic disease or disability may be exempted from being marked in PE class by their physician. CONCLUSION: The concept of DSE was widely accepted, but rarely used. It can be applied in various scenarios. In order to improve sports participation, a national standardized procedure should to be implemented.

Effects of exercise training on heart rate variability in children and adolescents with pulmonary arterial hypertension: a pilot study.

BACKGROUND: Pulmonary arterial hypertension (PAH) is often associated with cardiac autonomic dysfunction, and heart rate variability (HRV) as marker of cardiac autonomic function is even related to disease severity. Knowledge about the effects of physical activity on HRV is limited in these patients. We aimed to assess whether HRV parameters can be influenced by a supervised exercise training program and whether respective changes are related to levels of activity. METHODS: Six children and adolescents with moderate PAH (3 female, mean age 15.0±4.4 years; mean pulmonary to systemic arterial pressure ratio 0.5±0.2) performed supervised endurance and resistance training for 16 weeks. PAH-specific targeted medication remained unchanged during the study period. HRV was assessed before training and after 16 weeks of training by the use of ECG Move accelerometers. HRV indices included: standard deviation of normal-to-normal (NN) intervals (SDNN), square root of the mean of the sum of the squares of differences between successive NN-intervals (RMSSD), proportion of the number of pairs of successive normal-to-normal intervals that differ by more than 50 ms divided by total number of normal-to-normal interval (pNN50), and the Baevsky stress index (BSI) calculated by the histogram method. RESULTS: Before and after the training program, SDNN, RMSSD and pNN50 correlated with the level of physical activity. Mean values of SDNN, RMSSD and pNN50 did not change significantly due to the training program. Notably, activity level depending SDNN increased markedly after the exercise program (during activity +12.4%, at rest +6.6% and reclining +8.1%, Hedge's g of 0.28, 0.14 and 0.27, respectively). BSI decreased during activity due to the training program reflecting a reduction of stress level (Hedge's g -0.87 indicating an effect of clinical relevance). CONCLUSIONS: According to the experience of this pilot study a workout program of 16 weeks revealed an activity level dependent effect on parameters of autonomic cardiac function in children and adolescents with PAH. This mechanism might contribute to the positive effects of exercise training in patients with PAH.

Physical Activity Among Children With Congenital Heart Defects in Germany: A Nationwide Survey.

Objective: In children with congenital heart defects (CHD), a sedentary lifestyle should be avoided and usually WHO recommendations on physical activity (PA) are supposed to be followed. In order to obtain representative data of the actual amount of PA (and potential influencing factors) in children with CHD we performed a nationwide online survey. Methods: All patients aged 6-17 years registered in the German National Register for CHD were contacted by email and asked to participate in the survey using the comprehensive questionnaire of the "Motorik-Modul" from the German Health Interview and Examination Survey for Children and Adolescents (KiGGS), thus allowing the comparison with a representative age-matched subset of 3.385 participants of the KiGGS study. The questionnaire for CHD-patients was amended by specific questions regarding medical care, sports recommendations and PA restrictions. Results: Complete datasets of 1.198 patients (mean age of 11.6 ± 3.1 years) were available for evaluation. Compared to the reference group, CHD patients significantly less frequently reached the WHO recommended level of 60 min of daily PA (8.8 vs. 12%; p < 0.001). Enjoyment in sports was almost equally distributed across CHD and reference groups, and strongly correlated with the level of PA (r = 0.41; p < 0.001). Remarkably, 49.2% of children with complex CHD, 31.7% with moderate, and even 13.1% with simple CHD were advised by their physician to restrict PA. Conclusions: According to this nationwide survey, PA is markedly reduced in children with CHD. An important reason for this might be an unexpected high rate of physician-recommended restrictions on levels of PA.

Heart Rate Response During Treadmill Exercise Test in Children and Adolescents With Congenital Heart Disease.

Background: Impaired exercise capacity is a common feature of congenital heart disease (CHD). In adults with CHD, it has been shown that impaired heart rate response during exercise may contribute to exercise limitation. Systematic data in children and adolescents on this topic is limited. We therefore purposed to assess heart rate response during treadmill exercise testing in children and adolescents with CHD compared to healthy controls. Methods: One hundred and sixty three children and adolescents (103 with CHD, median age 15 years and 60 age-matched controls) performed cardiopulmonary exercise testing and were included in this study. Beyond peak oxygen consumption, increase in heart rate from resting level to peak exercise (heart rate reserve) and decrease of heart rate after peak exercise (heart rate recovery) were measured. Chronotropic index was defined as percentage of age predicted maximal heart rate reserve. According to data from adults on bicycle exercise, chronotropic incompetence was assumed for chronotropic index below 0.8. Results: While resting heart rate was similar between both groups, peak heart rate, heart rate reserve as well as chronotropic index were lower in the CHD group than in controls. Chronotropic index was lowest in patients with single ventricle hemodynamics and correlated with peak oxygen consumption. Heart rate recovery was impaired in the CHD group 1 and 2 min after peak exercise compared to controls and correlated with peak oxygen consumption. Chronotropic index below 0.8 was a relatively frequent finding even in the control group suggesting that the threshold of 0.8 appears inadequate for the identification of chronotropic incompetence using treadmill exercise testing in children. After normalizing to the 2.5th chronotropic index percentile of the control group we obtained a chronotropic incompetence threshold of 0.69. Conclusion: As an adjunct to measurement of peak oxygen consumption, heart rate response to exercise appears to be a physiologically important diagnostic parameter in children and adolescents with CHD. However, interpretation of heart rate response needs to consider specific age characteristics and the mode of exercise test. Our data may help future studies on chronotropic incompetence using treadmill ergometer protocols in children and adolescents.

Impact of the Right Ventricular Sokolow-Lyon Index in Children with Idiopathic Pulmonary Arterial Hypertension.

Right ventricular (RV) hypertrophy is regarded as the adaptation on chronic RV pressure load in pulmonary hypertension. As the RV Sokolow-Lyon index (RVSLI) is an electrocardiographic marker of RV hypertrophy, we hypothesized that RVSLI might be able to reflect RV pressure load. Therefore, the purpose of this study was to characterize the diagnostic impact of the RVSLI in children with idiopathic pulmonary arterial hypertension (IPAH) in order to assess disease severity and to evaluate its value for the prediction of worse outcome. Forty-two children (aged 3-17 years) with IPAH were included in this retrospective study. The follow-up after baseline examination was 59 ± 17 months. Receiver-operating characteristic (ROC) curves and Kaplan-Meier analysis were used to discriminate a cut-off value of RVSLI and to assess its predictive value regarding morbidity and mortality. In 12/42 patients (29%) severe cardiovascular events (defined as death, lung transplantation, or Potts shunt) were observed (time to event 20 ± 22 months). Patients with an event showed higher RVSLI values (3.6 ± 1.2 mV vs. 2.6 ± 1.6 mV; p < 0.05). ROC analysis discriminated an RVSLI of 2.1 as the best cut-off value (area under the ROC curve: 0.79, sensitivity: 0.91, specificity: 0.70, p < 0.05) to detect patients with high-risk PAH (mPAP/mSAP ratio > 0.75). Relative risk for a severe event with an index > 2.1 mV was 1.76 (95% CI 1.21-3.20). Relative risk for death with RVSLI > 2.1 mV was 2.01 (95% CI 1.61-4.80). Our study demonstrates a strong relationship between RVSLI and disease severity in children with IPAH. An RVSLI > 2.1 mV at the time of first diagnosis is a predictor for patients at risk for cardiac events. As an adjunct to the usual diagnostic assessment this parameter may therefore contribute to the initial prognostic estimation.

Home Exercise Training in Children and Adolescents with Pulmonary Arterial Hypertension: A Pilot Study.

Pulmonary arterial hypertension (PAH) is often associated with impaired exercise capacity. It has been shown that supervised training can improve exercise capacity in adult patients with PAH. The objective of this prospective study was to assess the feasibility of a home exercise training program in children with PAH. Nine children and adolescents (mean age 15.2 ± 3.8 years) with low-risk PAH (defined as mean pulmonary to systemic arterial pressure ratio <0.75; WHO functional class I and II) performed home-based exercise training for 16 weeks. Cardiopulmonary exercise testing and health-related quality of life was evaluated before and after 16 weeks of training. The amount of training at home and patients' well-being was supervised by periodical phone calls and online-questionnaires. Home exercise training was well tolerated in all patients, and no adverse events occurred. After 16 weeks of training, patients significantly improved their exercise capacity [treadmill running distance increased from 589.5 ± 153.9 to 747.9 ± 209.2 m (p = 0.036)]. Oxygen consumption at the anaerobic threshold increased from 1307.8 (±417) to 1406.4 (±418) ml (p = 0.028). Chronotropic index improved from 0.77 ± 0.12 to 0.82 ± 0.11 (p = 0.004) and was slightly related to the increase in running distance (r = 0.62; p = 0.07). Home exercise training is feasible in children and adolescents with low-risk PAH, and the preliminary results of this pilot study indicate beneficial effects. The observed increase in exercise capacity was accompanied by an improved chronotropic competence and increased oxygen consumption at the anaerobic threshold. Future research is needed to investigate the safety and efficacy of home exercise training in a larger population of children with PAH including also patients in WHO functional class III or IV.

Heart Rate Variability is Related to Disease Severity in Children and Young Adults with Pulmonary Hypertension.

BACKGROUND: Pulmonary hypertension (PH) is frequently associated with an increase in sympathetic tone. This may adversely affect cardiac autonomic control. Knowledge about the clinical impact of autonomic dysfunction in patients with PH is limited. We aimed to assess whether parameters of heart rate variability (HRV) are related to disease severity in children with PH. METHODS: Parameters of HRV [SDNN, standard deviation of normal-to-normal intervals and SDANN, standard deviation of mean values for normal-to-normal intervals over 5 min] were determined from Holter electrocardiograms of 17 patients with PH without active intracardial shunt (10 female, mean age 12.8 ± 8.7 years). Patients were allocated to two groups according to their disease severity: patients with moderate PH [ratio of pulmonary to systemic arterial pressure (PAP/SAP ratio) < 0.75] (n = 11), patients with severe PH (PAP/SAP ratio > 0.75) (n = 6). An additional group of five adolescents with Eisenmenger syndrome (PAP/SAP ratio 1.13 ± 0.36) was included. RESULTS: Children with severe PH had significantly lower values of HRV [SDNN (73.8 ± 21.1 vs. 164.9 ± 38.1 ms), SDANN (62.2 ± 19.0 vs. 139.5 ± 33.3 ms)] compared to patients with moderate PH (p = 0.0001 for all). SDNN inversely correlated with ratio of PAP/SAP of PH patients without shunt (r = -0.82; p = 0.0002). Eisenmenger patients showed no significant difference of HRV [SDNN 157.6 ± 43.2 ms, SDANN 141.2 ± 45.3 ms] compared to patients with moderate PH without shunt (p > 0.05 for all). CONCLUSION: According to our results, children with severe PH may have alterations in HRV. Since HRV appears to be related to disease severity, it may therefore serve as an additional diagnostic marker of PH. Remarkably, although Eisenmenger patients have systemic pulmonary arterial pressures, they seem to have preserved HRV, which might reflect a more favorable autonomic adaptation.