Cardiopulmonary Exercise Test and Daily Physical Activity in Pediatric Congenital Heart Disease: an Exploratory Analysis.

Sedentary lifestyle is reported to be associated with diminished exercise capacity, resulting in increased cardiovascular risk in adults with congenital heart disease (CHD). This cross-sectional study examined the association between objectively measured physical activity (PA) and exercise capacity in children and adolescents with CHD. Therefore, 107 patients (aged 13.0 ± 2.7 years, 41 girls) with various CHD performed a cardiopulmonary exercise test to quantify their peak oxygen uptake (peakV'O2). Moderate to vigorous PA (MVPA) and daily step count were assessed using Garmin vivofit jr. (Garmin, Germany) for 7 consecutive days. For association between PA and submaximal exercise capacity, Spearman correlation was performed. Patients with CHD showed almost normal values compared with the reference (79.5 ± 17.2% [31.6 to 138.1] %peakV'O2 predicted), with roughly normal ventilatory anerobic thresholds (50.6 ± 14.0% [20.3 to 97.9] % oxygen uptake at ventilatory anaerobic threshold [VATV'O2]). Step counts are below the recommendations (9,304 ± 3,792 steps/day [1,701 to 20,976]), whereas MVPA data are above the recommendations for children with ≥60 min/day (83.6 ± 34.6 min/day [10.1 to 190.9]). The Spearman rho showed significant positive correlations to VATV'O2 (r = 0.353, p <0.001) and %VATV'O2 (r = 0.307, p = 0.001), with similar results regarding MVPA (VATV'O2: r = 0.300, p = 0.002 and %VATV'O2: r = 0.270, p = 0.005). In conclusion, submaximal exercise capacity and PA correlate positively, making both assessments relevant in a clinical setting: PA in the context of cardiovascular prevention and peakV'O2 as the strongest predictor for morbidity and mortality.

KIJANI App to Promote Physical Activity in Children and Adolescents: Protocol for a Mixed Method Evaluation.

BACKGROUND: The prevalence of physical inactivity among children and adolescents is alarmingly high despite the well-documented and comprehensive benefits of regular physical activity (PA). Therefore, PA promotion should start early in childhood and adolescence. Although reducing recreational screen time in children and adolescents is an urgent concern, digital approaches have the potential to make activity promotion attractive and age appropriate for the target group. KIJANI is a mobile app approach to promote PA in children and adolescents via gamification and augmented reality. OBJECTIVE: This study protocol aims to describe the KIJANI intervention in detail, as well as the evaluation approach. METHODS: KIJANI is based on the concept that virtual coins can be earned through PA, for example, in the form of a collected step count. With these coins, in turn, blocks can be bought, which can be used to create virtual buildings and integrate them into the player's real-world environment via augmented reality. PA of users is detected via accelerometers integrated into the smartphones. KIJANI can be played at predefined play locations that were comprehensively identified as safe, child-friendly, and attractive for PA by the target group in a partner project. The evaluation process will be divided into 2 different stages. The phase-I evaluation will be a mixed methods approach with one-on-one semistructured interviews and questionnaires to evaluate the user experience and receive feedback from the target group. After the implementation of results and feedback from the target group, the phase-II evaluation will proceed in the form of a 2-arm randomized controlled trial, in which the effectiveness of KIJANI will be assessed via objectively measured PA as well as questionnaires. RESULTS: The study received ethical approval from the ethical board of the Technical University of Munich. Participants for the phase-I evaluation are currently being recruited. CONCLUSIONS: The study will help to determine the efficacy, applicability, and user experience of a gamified activity promotion application in children and adolescents. Overall, digital health approaches provide easy and wide reachability at low cost and are age appropriate and attractive for the target group of adolescents. Strategies have to be developed to apply digital health approaches in the best possible way for activity promotion. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/55156.

KIJANI: Designing a Physical Activity Promoting Collaborative Augmented Reality Game.

BACKGROUND: There is an increased need for physical activity among children and adolescents. KIJANI, a mobile augmented reality game, is designed to increase physical activity through gamified exercises. OBJECTIVES: The primary aim of this study is to get feedback on the design and implementation of potentially physical activity-increasing features in KIJANI. METHODS: A mixed-method study (n=13) evaluates newly implemented game design features quantitatively through measuring physical activity and qualitatively through participant feedback. RESULTS: Preliminary results are limited and need further studies. Participants' feedback shows a positive trend and highlights the game's potential effectiveness. CONCLUSION: KIJANI shows potential for increasing physical activity among children and adolescents through gamified exercise. Future work will refine the game based on user feedback and findings presented in related work. The game's long-term impact is to be explored.

High-sensitive troponin T and N-terminal pro-B-type natriuretic peptide independently predict survival and cardiac-related events in adults with congenital heart disease.

AIMS: High-sensitive troponin T (hs-TnT), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and C-reactive protein (CRP) are established prognostic biomarkers for cardiovascular (CV) morbidity and mortality and frequently used in symptomatic and/or hospitalized adults with congenital heart disease (ACHD). Their prognostic value in clinically stable ACHD has not yet been well established. This study investigates the predictive value of hs-TnT, NT-proBNP, and CRP for survival and CV events in stable ACHD. METHODS AND RESULTS: In this prospective cohort study, 495 outpatient ACHD (43.9 ± 10.0 years, 49.1% female) underwent venous blood sampling including hs-TnT, NT-proBNP, and CRP. Patients were followed up for survival status and the occurrence of CV events. Survival analyses were performed with Cox proportional hazards regression analysis and Kaplan-Meier curves. During a mean follow-up of 2.8 ± 1.0 years, 53 patients (10.7%) died or reached a cardiac-related endpoint including sustained ventricular tachycardia, hospitalization with cardiac decompensation, ablation, interventional catheterization, pacer implantation, or cardiac surgery. Multivariable Cox regression revealed hs-TnT (P = 0.005) and NT-proBNP (P = 0.018) as independent predictors of death or cardiac-related events in stable ACHD, whilst the prognostic value of CRP vanished after multivariable adjustment (P = 0.057). Receiver-operator characteristic curve analysis identified cut-off values for event-free survival of hs-TnT ≤9 ng/L and NT-proBNP ≤200 ng/L. Patients with both increased biomarkers had a 7.7-fold (confidence interval 3.57-16.40, P < 0.001) higher risk for death and cardiac-related events compared with patients without elevated blood values. CONCLUSION: Subclinical values of hs-TnT and NT-proBNP are a useful, simple, and independent prognostic tool for adverse cardiac events and survival in stable outpatient ACHD. REGISTRATION: German Clinical Trial Registry DRKS00015248.

Longitudinal development of central SBP in children with congenital heart disease.

AIMS: Central SBP (cSBP) was shown to be increased already in children with congenital heart disease (CHD). However, its development over time has not yet been investigated. The aim of this study was to evaluate the natural course of cSBP over time from longitudinal assessment in children with CHD. METHODS: In this longitudinal study, 306 children and adolescents (11.3 ±â€Š2.9 years, 34% girls) with various CHD were prospectively examined from July 2014 to May 2022. Over a mean follow-up length of 30.1 ±â€Š18.9 months, 467 follow-up assessments have been conducted. cSBP was noninvasively assessed by oscillometric measurement via Mobil-O-Graph. A linear mixed effect model was performed to examine the course of cSBP. RESULTS: cSBP increased significantly over time by 1.22 mmHg per year of age (P < 0.001). The longitudinal increase in cSBP over time remained significant when including sex (b = 0.68, P < 0.001), BMI (b = 1.12, P < 0.001), hypertensive medication (b = 1.13, P < 0.001), disease severity (b = 1.04, P < 0.001), and CHD type (b = 3.74, P = 0.03) in the model. Patients with transposition of the great arteries (TGA) after arterial switch had a significantly higher cSBP increase over time (b = 1.78, P < 0.001). The longitudinal cSBP increase was significantly higher in obese CHD children (b = 2.52, P = 0.005) and in boys (b = 0.85, P < 0.001). CONCLUSION: This study shows a longitudinal increase in cSBP in children with CHD. Whether observed trajectories of cSBP are normal or abnormal needs to be investigated in further studies. Monitoring of the vascular function with a special focus on patients with TGA and obese CHD children seems indicated.

Resilience in children with congenital heart disease: a comparative study with health counterparts.

OBJECTIVE: Resilience is a complex, yet rather unexplored topic in patients with congenital heart disease (CHD). The goal of this study was to assess and compare resilience in children with CHD with healthy controls during the COVID-19 pandemic. DESIGN AND PATIENTS: From June 2020 to June 2021, 124 children with various CHDs (14.6±2.1 years, 49 girls) and 124 matched healthy controls (14.8±2.0 years, 49 girls) completed the Resilience Scale-11 short version. RESULTS: Resilience was significantly reduced in children with CHD compared with healthy controls (CHD: 59.0±10.0 vs healthy controls: 64.4±6.5, p<0.001). That reduction was prominent in all CHD subgroups except those with left heart obstruction (aortic stenosis and coarctation of the aorta) and patients with transposition of the great arteries. Complex CHD had the lowest resilience of 57.6±8.4 (p<0.001) after adjusting for age and sex according to group differences. There was no difference between native CHD and CHD with open-heart surgery (native: 59.5±12.2 vs surgery: 58.8±9.3, p=0.758). CONCLUSIONS: Resilience was reduced in children and adolescents with CHD compared with healthy peers during the COVID-19 pandemic. Children with complex severity appeared to be particularly affected. These findings emphasise continued efforts to provide a holistic and multidisciplinary approach in medical aftercare of these patients and their families.

Digital Health Nudging to increase physical activity in pediatric patients with congenital heart disease: A randomized controlled trial.

BACKGROUND: Digital nudging is a modern e-health approach to increase physical activity (PA) in younger age groups. As activity promotion is particularly important in adolescents with congenital heart disease (CHD) this randomized-controlled trail examines if Digital Health Nudging via daily smartphone messages increases PA, activity-related self-efficacy (ArSE) and health-related quality of life (HrQoL) in adolescents with CHD. METHODS: From May 2021 to April 2022, 97 patients (15.1 ± 2.0 years, 50% girls) with moderate or severe CHD were randomly allocated 1:1 to intervention (IG) or control group (CG). Daily PA was objectively assessed in minutes of moderate-to-vigorous PA (MVPA) by the wearable "Garmin Vivofit jr. 2" over the entire study period. The IG received daily smartphone messages based on Bandura's social cognitive theory on the subject of PA over a period of 12-weeks. RESULTS: According to the linear mixed model, the change of MVPA over the study period did not significantly differ between IG and CG when taking baseline MVPA into account (b = 0.136, 95%-CI [-0.355; 0.627], P = .587). Activity level was comparably high and showed only minor variability in both groups with 73.7 [62.3; 78.8] min/day in IG and 78.4 [66.6; 93.9] min/d in CG throughout the whole 12-weeks. Emotional well-being significantly increased over the study period in the IG (IG: Δ1.60 [-0.2; 6.3] vs CG: Δ0.0 [-12.5; 6.3], P = .043), but not total HrQoL (P = .518) and ArSE (P = .305). CONCLUSIONS: 12-weeks, of Digital Health Nudging did not increase PA, but improved feelings of emotional well-being in adolescents with CHD. TRIAL REGISTRATION: Clinical Trials Identifier NCT04933786.

Abnormalities in pulmonary function and volumes in patients with CHD: a systematic review.

BACKGROUND: Lung function and cardiac function are naturally correlated by sharing the thoracic cage and handling the whole cardiac output sequentially. However, lung function studies are rare in patients with CHD, although results worthy of investigation could be expected. This review summarises existing studies with the lung function parameters (spirometry and body plethysmography) in CHD patients during the last decade. METHODS: A systematic review was performed in the relevant database (PubMed, Cochrane, and Scopus) in studies including paediatric and adult patients with CHD where lung parameters (spirometry, body plethysmography) were investigated from January 2010 to December 2020. Two independent reviewers evaluated the studies according to the Study Quality Assessment Tool for Observational Cohort and Cross-Sectional Studies of the National Heart, Lung, and Blood Institute. RESULTS: Eight studies investigated patients with Fontan palliation including 704 patients (306 female). Four studies included patients after repaired tetralogy of Fallot examining 219 patients (103 female), with one study using double. Further six studies included 3208 (1324 female) children and adults with various CHDs. Overall, four studies were categorised as "good", ten as "fair", and four as "poor". While the measurements were consistently standardised, references to calculate %predicted differed substantially across all studies. All evaluated studies showed reduced forced vital capacity in the majority of CHD patients. CONCLUSIONS: Many CHD patients have a reduced forced vital capacity independent of their underlying defect. Spirometry should not only follow a standardised measure according to ATS (update 2019) but also stick to the 2012 GLI reference values.

Heart-Focused Anxiety Is Prevalent in Adults With Congenital Heart Disease and Associated With Reduced Exercise Capacity.

PURPOSE: Psychological distress and anxiety are increased among adults with congenital heart disease (CHD). However, heart-focused anxiety (HFA) and its impact on peak oxygen uptake (V˙ o2peak ) have not yet been studied in this patient cohort. This study investigates HFA in adults with CHD and whether HFA is associated with V˙ o2peak in these patients. METHODS: In this cross-sectional study, HFA was assessed in 984 adults with various CHD (34.2 ± 11.2 yr, 44% female) by the cardiac anxiety questionnaire. In 708 of those (33.5 ± 10.6 yr, 42% female), additional cardiopulmonary exercise testing was performed to assess exercise capacity by measurement of V˙ o2peak . RESULTS: Compared with the general population, this adult CHD population showed higher HFA in the total cardiac anxiety questionnaire score ( z score; 1.32 ± 1.17; P < .001), as well as in the three subdomains anxiety, avoidance, and attention (all P < .001). The higher the HFA, the lower was the V˙ o2peak % predicted ( r = -0.280, P < .001) independent of CHD diagnosis, CHD severity, and surgical status. The V˙ o2peak % predicted significantly differed between patients with no (85.9 ± 18.4% predicted), slight (80.4 ± 18.4% predicted), and severe HFA (74.2 ± 18.4% predicted) independent of CHD diagnosis, CHD severity, and surgical status ( P < .001). CONCLUSION: This study shows that HFA is a common sequela in adults with CHD, and high HFA is associated with reduced V˙ o2peak . Psychological screening and counseling for HFA therefore seem indicated in the aftercare of adults with CHD.

Diminished Endothelial Function but Normal Vascular Structure in Adults with Tetralogy of Fallot.

The life expectancy of patients with Tetralogy of Fallot (ToF) has increased in recent years. As a result, other risk factors with later onset in life are in the focus of patient care. Endothelial function is an early indicator of cardiovascular risk and was investigated along further structural vessel properties. A total of 17 patients (41.7 ± 7.1 years, 8 women) with Tetralogy of Fallot were 1:2 matched for sex with 34 (38.9 ± 8.1 years, 16 women) healthy volunteers. Participants received an assessment of their endothelial function and a structural assessment of the aorta. Patients with ToF showed a reduced endothelial function determined by reactive hyperaemia index after adjusting for age, weight and height (ToF: 1.55 ± 0.31 vs. controls: 1.84 ± 0.47; p = 0.023). No differences in carotid intima-media thickness (cIMT) between the ToF and healthy controls (ToF: 0.542 ± 0.063 mm vs. controls: 0.521 ± 0.164 mm; p = 0.319) were found. Patients with ToF had reduced vascular function compared to healthy subjects. As the structural component is not affected, endothelial dysfunction seems not to have yet manifested itself as a morphological change. Nevertheless, long-term management of these patients should include vascular parameters.

Overweight and Obesity in Patients with Congenital Heart Disease: A Systematic Review.

BACKGROUND: Overweight and obesity have become a major public health concern in recent decades, particularly in patients with chronic health conditions like congenital heart disease (CHD). This systematic review elaborates on the prevalence and the longitudinal development of overweight and obesity in children and adults with CHD. METHODS: A systematic literature search was conducted in PubMed, Cochrane, and Scopus from January 2010 to December 2020 on overweight and obesity prevalence in children and adults with CHD. RESULTS: Of 30 included studies, 15 studies evaluated 5680 pediatric patients with CHD, 9 studies evaluated 6657 adults with CHD (ACHD) and 6 studies examined 9273 both pediatric patients and ACHD. Fifteen studies received the quality rating "good", nine studies "fair", and six studies "poor". In children with CHD, overweight prevalence was between 9.5-31.5%, and obesity prevalence was between 9.5-26%; in ACHD, overweight prevalence was between 22-53%, and obesity was between 7-26%. The prevalence of overweight and obesity was thereby similar to the general population. Overweight and obesity have been shown to increase with age. CONCLUSION: The prevalence of overweight and obesity in children and adults with CHD is similar to the general population, demonstrating that the growing obesity pandemic is also affecting the CHD population.

Association between Objectively Measured Physical Activity and Arterial Stiffness in Children with Congenital Heart Disease.

BACKGROUND: The association between physical activity (PA) and arterial stiffness is particularly important in children with congenital heart disease (CHD) who are at risk for arterial stiffening. The aim of this study was to examine the association between objectively measured PA and arterial stiffness in children and adolescents with CHD. METHODS: In 387 children and adolescents with various CHD (12.2 ± 3.3 years; 162 girls) moderate-to-vigorous PA (MVPA) was assessed with the "Garmin vivofit jr." for 7 consecutive days. Arterial stiffness parameters including pulse wave velocity (PWV) and central systolic blood pressure (cSBP) were non-invasively assessed by oscillometric measurement via Mobil-O-Graph®. RESULTS: MVPA was not associated with PWV (ß = -0.025, p = 0.446) and cSBP (ß = -0.020, p = 0.552) in children with CHD after adjusting for age, sex, BMI z-score, peripheral systolic blood pressure, heart rate and hypertensive agents. Children with CHD were remarkably active with 80% of the study population reaching the WHO recommendation of average 60 min of MVPA per day. Arterial stiffness did not differ between low-active and high-active CHD group after adjusting for age, sex, BMI z-score, peripheral systolic blood pressure, heart rate and hypertensive agents (PWV: F = 0.530, p = 0.467; cSBP: F = 0.843, p = 0.359). CONCLUSION: In this active cohort, no association between PA and arterial stiffness was found. Longer exposure to the respective risk factors of physical inactivity might be necessary to determine an impact of PA on the vascular system.

Prognostic value of non-acute high sensitive troponin-T for cardiovascular morbidity and mortality in adults with congenital heart disease: A systematic review.

BACKGROUND: Elevation of high-sensitivity troponin-T (hs-TnT) is linked to cardiovascular morbidity and mortality. However, its prognostic value for survival and cardiovascular events and its relation to clinical characteristics and cardiac function parameters in clinically asymptomatic adults with congenital heart disease (ACHD) needs further exploration. METHODS: A systematic literature search was performed in PubMed and Cochrane from 2010 to May 2020 for hs-TnT as a prognostic marker in ACHD. Three independent reviewers evaluated the articles according to the Study Quality Assessment Tool for Observational Cohort and Cross-Sectional Studies of the National Heart, Lung, and Blood Institute. Overall, eight studies with a total of 2162 ACHD patients (18-63 years) were included. RESULTS: Hs-TnT level was elevated in 8-26% of asymptomatic ACHD. The follow-up for all-cause mortality and cardiovascular events ranged from 3.0 to 5.6 years and in 8-38% of the participants cardiac endpoints were reached. Throughout the included studies, elevated hs-TnT was found to be an independent predictor for survival and heart failure in stable ACHD. Serial hs-TnT measurement was found to be beneficial over single measurement. Hs-TnT levels were correlated with male sex, higher age, and higher New York Heart Association class and associated with several cardiac dysfunction parameters. CONCLUSION: More scientific research investigating the prognostic value of hs-TnT in stable ACHD is needed and the clinical relevance to guide aftercare has still to be determined.