Heart rate dynamics and asymmetry during sympathetic activity stimulation and post-stimulation recovery in ski mountaineers-a pilot exploratory study.

There is a lack of studies on non-linear heart rate (HR) variability in athletes. We aimed to assess the usefulness of short-term HR dynamics and asymmetry parameters to evaluate the neural modulation of cardiac activity based on non-stationary RR interval series by studying their changes during sympathetic nervous system activity stimulation (isometric handgrip test) and post-stimulation recovery in professional ski mountaineers. The correlation between the changes in the parameters and the respiratory rate (RespRate) and also the duration of the career was analyzed. Short-term (5 min) and ultra-short-term (1 min) rates of patterns with no variations (0V), number of acceleration runs of length 1 (AR1), and short-term Porta's Index were greater, whereas Guzik's Index (GI) was smaller during sympathetic stimulation compared to rest. GI increased and the number of AR1 decreased during recovery. Greater increases in GI and RMSSD were associated with greater decreases in RespRate during recovery. Greater increases in RespRate from rest to short-term sympathetic stimulation were associated with greater increases in 0V (Max-min method) and AR1 but also with greater decreases in decelerations of short-term variance and accelerations and decelerations of long-term variance. Greater increases in 0V (Max-min method) and number of AR1 during sympathetic stimulation were associated with a shorter career duration. Greater decreases in these parameters during recovery were associated with a longer career duration. Changes in measures of HR dynamics and asymmetry, calculated based on short-term non-stationary RRi time series induced by sympathetic stimulation and post-stimulation recovery, reflected sympathovagal shift and were associated with condition-related alterations in RespRate and career duration in athletes who practice ski mountaineering.

A MYH7 variant in a five-generation-family with hypertrophic cardiomyopathy.

Background: Hypertrophic cardiomyopathy (HCM) is a genetic condition with a prevalence of 1:500-1:3 000. Variants in genes encoding sarcomeric proteins are mainly responsible for the disease. MYH7 gene encoding a myosin heavy chain beta, together with MYPBC3 gene are the two most commonly affected genes. The clinical presentation of this disease varies widely between individuals. This study aims to report a variant of MYH7 responsible for HCM in a five-generation family with a history of cardiac problems. Methods: The diagnosis was established according to the European Society of Cardiology HCM criteria based on two-dimensional Doppler echocardiography or cardiovascular magnetic resonance. Genetic analysis was performed using next-generation-sequencing and Sanger method. Results: The medical history of the presented family began with a prenatal diagnosis of HCM in the first child of a family with previously healthy parents. Five generations of the family had a long history of sudden cardiac death and cardiac problems. A NM_000257.4:c.2342T>A (p.Leu781Gln) variant was detected in the MYH7 gene. It was heterozygous in the proband and in all affected individuals in a large family. The variant was present in 10 affected members of the family, and was absent in 7 members. The clinical course of the disease was severe in several members of the family: three family members died of sudden cardiac death, one patient required heart transplantation, three underwent septal myectomy, and three required implantable cardioverter defibrillator (ICD) implantation. Conclusion: Herein, we report a MYH7 variant responsible for HCM. Familial HCM is inherited primarily in autosomal dominant mode, which is in accordance with our study. However, the presented family showed a broad clinical spectrum of HCM. Out of 10 family members with positive genetic testing 8 had severe presentation of the disease and 2 had a mild phenotype. This suggests that the severity of the disease may depend on other factors, most likely genetic.

The discovery of a data-driven causal diagram of sport participation in children and adolescents with heart disease: a pilot study.

The interventions aimed at improving the levels of physical activity (PA) in children and adolescents diagnosed with heart disease did not produce the expected outcomes. Safe participation in sport activities proposed based on actual recommendations could be a solution to promote PA in this population. The aims of this study were to discover a causal diagram of sport participation in children and youth with heart disease and establish the factors that affect and are affected thereof through the use of questionnaires. Furthermore, the study aims to qualitatively assess the reliability of the constructed diagram in comparison with existing medical knowledge. The Greedy Fast Causal Inference method was employed to conduct a data-driven search of the directed acyclic graph that represents the causal relationships within the provided observational data. This causal discovery was performed using the Tetrad software. The analysis involved a cohort of 121 Caucasian patients (50 females) diagnosed with heart disease. The age range of the patients included in the study was 8-17 years. The study findings indicate that the participants engaged in sports presented significantly higher values of health-related quality of life (QoL) and motives for participating in physical and leisure activities. Age appears to be a cause of sport participation. Sport participation appears to be a cause of participation in physical education classes, which in turn appears to be a cause of higher enjoyment. Higher enjoyment appears to be a cause of other motives for participating in physical and leisure activities, as well as a higher score in terms of physical health. The causal diagram provided a graphical representation of the causal relationship between sport participation and better QoL with potential confounders for children and adolescents with heart disease that nearly coincided with the existing literature. Clinical trials should be designed to validate clinical utility of the presented causal diagram.

Inter-Rater Reliability of the Polish Version of the Alberta Infant Motor Scale in Children with Heart Disease.

There is an urgent need for the systematic monitoring of motor and cognitive neurodevelopment and the evaluation of motor skill development in infants and children with heart disease. Familiarizing students and early graduates with the developmental care needed by these patients may help in the system-wide implementation of early motor screening in this population. The purpose of this study was to investigate the agreement between a last-year physiotherapy student and an experienced pediatric physiotherapist when applying the Polish version of the Alberta Infant Motor Scale (AIMS) to a heterogenous group of children with congenital heart defects. Agreement between raters was verified based on the observation of 80 (38 females) patients with heart disease aged 1-18 months using a Bland-Altman plot with limits of agreement and an intraclass correlation coefficient. The bias between raters for the total score for four age groups (0-3 months, 4-7 months, 8-11 months and 12-18 months) was between -0.17 and 0.22 (range: -0.54-0.78), and the ICC was between 0.875 and 1.000. Thus, a reliable assessment of motor development or motor skills using the Polish version of the AIMS can be performed in pediatric patients with heart defects by clinically inexperienced last-year physiotherapy students who are familiarized with the AIMS manual.

Three-Dimensional Echocardiography and Global Longitudinal Strain in Follow-Up After Multisystem Inflammatory Syndrome in Children: Six-Month, Single-Center, Prospective Study.

OBJECTIVE: To assess the potential long-term cardiac effects after multisystem inflammatory syndrome in children (MIS-C) with cardiovascular involvement in the acute phase. STUDY DESIGN: Our prospective study involved children consecutively diagnosed with MIS-C between October 2020 and February 2022 and followed 6 weeks and 6 months after the disease. In patients with severe cardiac involvement during the acute phase, an additional check-up after 3 months was scheduled. In all patients at all check-ups, 3-dimensional echocardiography and global longitudinal strain (GLS) were used to assess ventricular function. RESULTS: The study enrolled 172 children aged 1-17 years (median, 8 years). The means of ejection fraction (EF) and GLS for both ventricles were within normal limits after 6 weeks with no relationship with initial severity: left ventricular EF (LVEF) 60% (59%-63%), LV GLS -21.08% (-18.63% to -23.2%), right ventricular (RV) EF 64% (62%-67%), and RV GLS -22.8% (-20.5% to -24.5%). Further, statistically significant improvement of LV function was observed after 6 months-LVEF 63% (62%-65%) and LV GLS -22.55% (-21.05% to -24.25%; P < .05); however, RV function remained unchanged. The group with severe cardiac involvement showed LV function recovery pattern with no significant improvement between 6 weeks and 3 months after MIS-C, while still improving between 3 and 6 months after discharge. CONCLUSIONS: LV and RV function is within normal limits 6 weeks after MIS-C regardless of severity of cardiovascular involvement; LV function improves further between 6 weeks and 6 months after the disease. The long-term prognosis is optimistic with full recovery of cardiac function.

Diastolic function evaluation in children with ventricular arrhythmia.

Premature ventricular contractions (PVC) are frequently seen in children. We evaluated left ventricular diastolic function in PVC children with normal left ventricular systolic function to detect whether diastolic function disturbances affect physical performance. The study group consisted of 36 PVC children, and the control group comprised 33 healthy volunteers. Echocardiographic diastolic function parameters such as left atrial volume index (LAVI), left atrial strains (AC-R, AC-CT, AC-CD), E wave, E deceleration time (Edt), E/E' ratio, and isovolumic relaxation time (IVRT) were measured. In the cardiopulmonary exercise test (CPET), oxygen uptake (VO2 max) was registered. Evaluation of diastolic function parameters revealed statically significant differences between the patients and controls regarding Edt (176.58 ± 54.8 ms vs. 136.94 ± 27.8 ms, p < 0.01), E/E' (12.6 ± 3.0 vs. 6.7 ± 1.0, p < 0.01), and IVRT (96.6 ± 19.09 ms. vs. 72.86 ± 13.67 ms, p < 0.01). Left atrial function was impaired in the study group compared to controls: LAVI (25.3 ± 8.2 ml/m2 vs. 19.2 ± 7.5 ml/m2, p < 0.01), AC-CT (34.8 ± 8.6% vs. 44.8 ± 11.8%, p < 0.01), and AC-R-(6.0 ± 4.9% vs. -11.5 ± 3.5%, p < 0.01), respectively. VO2 max in the study group reached 33.1 ± 6.2 ml/min/kg. A statistically significant, moderate, negative correlation between VO2 max and E/E' (r = -0.33, p = 0.02) was found. Left ventricular diastolic function is impaired and deteriorates with the arrhythmia burden increase in PVC children. Ventricular arrhythmia in young individuals may be related to the filling pressure elevation and drive to exercise capacity deterioration.

Validity of the Pneumonitor for RR intervals acquisition for short-term heart rate variability analysis extended with respiratory data in pediatric cardiac patients.

BACKGROUND: Breathing pattern alterations change the variability and spectral content of the RR intervals (RRi) on electrocardiogram (ECG). However, there is no method to record and control participants' breathing without influencing its natural rate and depth in heart rate variability (HRV) studies. AIM: This study aimed to assess the validity of the Pneumonitor for acquisition of short-term (5 minutes) RRi in comparison to the reference ECG method for analysis of heart rate (HR) and HRV parameters in the group of pediatric patients with cardiac disease. METHODS: Nineteen patients of both sexes participated in the study. An ECG and Pneumonitor were used to record RRi in 5-minute static rest conditions, the latter also to measure the relative tidal volume and respiratory rate. The validation comprised Student's t-test, Bland-Altman analysis, intraclass correlation coefficient, and Lin's concordance correlation. The possible impact of respiratory activity on the agreement between ECG and the Pneumonitor was also assessed. RESULTS: An acceptable agreement for the number of RRi, mean RR, hazard ratio (HR), and HRV measures calculated based on RRi acquired using the ECG and Pneumonitor was presented. There was no association between the breathing pattern and RRi agreement between devices. CONCLUSIONS: The Pneumonitor might be considered appropriate for cardiorespiratory studies in the group of pediatric cardiac patients in rest condition.

Heart Rate Variability in Individuals with Down Syndrome: A Scoping Review with Methodological Considerations.

Individuals with Down syndrome (DS) present similar heart rate variability (HRV) parameters at rest but different responses to selected movement maneuvers in comparison to individuals without DS, which indicates reduced vagal regulation. The present study undertakes a scoping review of research on HRV in individuals with DS, with special attention paid to the compliance of the studies with standards and methodological paper guidelines for HRV assessment and interpretation. A review was performed using PubMed, Web of Science and CINAHL databases to search for English language publications from 1996 to 2020 with the MESH terms "heart rate variability" and "down syndrome", with the additional inclusion criteria of including only human participants and empirical investigations. From 74 studies, 15 were included in the review. None of the reviewed studies met the recommendations laid out by the standards and guidelines for providing the acquisition of RR intervals and necessary details on HRV analysis. Since authors publishing papers on this research topic do not adhere to the prescribed standards and guidelines when constructing the methodology, results of the research papers on the topic are not directly comparable. Authors need to design the study methodology more robustly by following the aforementioned standards, guidelines and recommendations.

Cardiopulmonary capacity is reduced in children with ventricular arrhythmia.

BACKGROUND: Premature ventricular contractions (PVCs) are frequently seen in children and are considered benign. A substantial group of adolescents with PVCs complain about a broad range of clinical symptoms, including low exertion tolerance. OBJECTIVE: The purpose of this study was to evaluate prospectively whether ventricular arrhythmia affects physical performance in adolescents with normal left ventricular function, using a cardiopulmonary exercise test (CPET) and evaluating the electrocardiographic (ECG) characteristics of patients with PVCs with regard to exercise capacity. METHODS: The study group consisted of 49 children with PVCs and normal left ventricular function. The control group consisted of 36 healthy volunteers. Standard ECG, 24-hour Holter ECG, and CPET were performed. PVCs were analyzed for QRS duration, bundle branch block pattern, QRS axis, and coupling interval (CInt). For CPET, heart rate (HR), oxygen uptake (VO2max), predicted VO2max, and VO2max expressed as a percentage of the predicted value (%VO2) were measured. RESULTS: In 37 patients (76%), arrhythmia subsided during exercise. Patients achieved lower VO2max (32.9 ± 6.3 mL/min/kg) than controls (40.4 ± 6.7 mL/min/kg; P <.01). %VO2 was 71.0 ± 13.7 in patients and 79.3 ± 12.2 in controls (P <.01). Exercise HR at which PVCs subsided correlated with VO2max (r = 0.3; P = .07). Patients with persisting arrhythmia performed worse than those in whom arrhythmia subsided during exercise (VO2max, P <.01; %VO2,P <.01). No correlation between QRS and CInt parameters and VO2max was observed. CONCLUSION: Patients with PVCs have lower aerobic capacity than their healthy peers. Further worsening of exercise capacity is present when PVCs are preserved during effort.

Electrocardiographic Parameters Associated with Adverse Outcomes in Children with Cardiomyopathies.

Cardiomyopathies have a low prevalence in children and thus may lead to malignant ventricular arrhythmias or the progression of heart failure, resulting in death. In adults, the QRS-T angle derived from ECG has been associated with adverse outcomes in patients with hypertrophic and dilated cardiomyopathies. We aimed to assess the electrocardiographic parameters, including QRS-T angle, associated with adverse cardiac events in children with cardiomyopathies. Forty-two children with cardiomyopathies were included in this study: 19 with dilated cardiomyopathy, 17 with hypertrophic cardiomyopathy, and 6 with left ventricular non-compaction. Additionally, 19 control subjects were recruited. In terms of ECG parameters, the QRS-T angle was significantly greater among patients with adverse outcomes compared to patients without the end points of the study (133° vs. 65°, p < 0.001). On Kaplan−Meier survival curves, QRS-T angle > 120°, increased serum concentrations of NT-proBNP and troponin I levels as well as greater NYHA or Ross scale were associated with the greatest risk of unfavorable outcome. The QRS-T angle appears to be a valuable component of 12-lead ECG interpretation, and might be helpful in outlining patients with the greatest cardiovascular risk. Additionally, serum biomarkers such as NT-proBNP (p = 0.003) and troponin (p < 0.001) are useful in outlining patients with the worst survival.

Causality in cardiorespiratory signals in pediatric cardiac patients.

Four different Granger causality-based methods - one linear and three nonlinear (Granger Causality, Kernel Granger Causality, large-scale Nonlinear Granger Causality, and Neural Network Granger Causality) were used for assessment and causal-based quantification of the respiratory sinus arrythmia (RSA) in the group of pediatric cardiac patients, based on the single-lead ECG and impedance pneumography signals (the latter as the tidal volume curve equivalent). Each method was able to detect the dependency (in terms of causal inference) between respiratory and cardiac signals. The correlations between quantified RSA and the demographic parameters were also studied, but the results differ for each method. Clinical relevance- The presented methods (among which NNGC seems to be the most valid) allow for quantification of RSA and study of dependency between tidal volume and RR intervals which may help to better understand association between respiratory and cardiovascular systems in different populations.

Subcutaneous Implantable Cardioverter Defibrillators for the Prevention of Sudden Cardiac Death: Pediatric Single-Center Experience.

BACKGROUND: The subcutaneous implantable cardioverter defibrillator (S-ICD) was developed as an alternative to the transvenous ICD, to prevent lead-related complications associated with the latter. The absence of intravascular or intracardiac components offers potential advantages to pediatric patients. AIMS: The aim of the study is to present an overview of our experience with S-ICDs in the pediatric center that, currently, has performed the largest number of implantations in children in Poland. METHODS: Retrospective analysis of data from medical history, qualification, implantation procedure, and S-ICD post-implantation observations in 11 pediatric patients were performed. RESULTS: S-ICDs were implanted in 11 patients, 8 boys and 3 girls, aged 12-17 years. The S-ICD was implanted for primary prevention in seven patients: four with hypertrophic cardiomyopathy (HCM), two with dilated cardiomyopathy (DCM), and one with arrhythmogenic right ventricular cardiomyopathy (ARVC). It was implanted for secondary prevention in four patients: two with sudden cardiac arrest (SCA) in the course of idiopathic ventricular fibrillation (IVF), one with long QT syndrome (LQTS) after probable SCA, and one with Brugada syndrome after SCA. In all patients, the device was implanted intramuscularly. One patient did not have a defibrillation test performed due to the presence of an intracardiac thrombus. In one patient, during screening, it was decided to implant an electrode on the right side of the sternum. There were no early or late complications with any of the procedures. So far, no inadequate discharges have been observed. CONCLUSIONS: Our results prove the efficacy of the S-ICD treatment option along with technically simple surgery, which supports its further and more widespread application in children.

Circadian Blood Pressure Profile in Pediatric Patients with Primary Hypertension.

Our study aimed to evaluate factors affecting circadian BP profile and its association with hypertension-mediated organ damage (HMOD) in pediatric patients with primary hypertension (PH). The study included 112 children (14.7 ± 2.1 age, 79 boys, 33 girls) with untreated PH. Non-dipping was defined as a nocturnal drop in systolic or diastolic BP (SBP, DBP) < 10%, and a nocturnal drop >20% was defined as extreme dipping. The nocturnal SBP drop was 10.9 ± 5.9 (%), and the DBP drop was 16.2 ± 8.5 (%). Non-dipping was found in 50 (44.6%) children and extreme dipping in 29 (25.9%) patients. The nocturnal SBP decrease correlated with BMI Z-score (r = −0.242, p = 0.010) and left ventricular mass index (LVMI) (r = −0.395, p = 0.006); diastolic DBP decrease correlated with augmentation index (AIx75HR) (r = 0.367, p = 0.003). Patients with a disturbed blood pressure profile had the highest LVMI (p = 0.049), while extreme dippers had the highest augmentation index (AIx75HR) (p = 0.027). Elevated systolic and diastolic BP dipping were risk factors for positive AIx75HR (OR 1.122 95CI (1.009−1.249) and OR 1.095 95CI (1.017−1.177). We concluded that disturbed circadian BP profile was common in children with PH and should not be considered a marker of secondary hypertension. A disturbed circadian BP profile may be associated with higher body weight. In pediatric patients with PH, non-dipping is associated with increased left ventricular mass, and extreme dipping may be a risk factor for increased arterial stiffness.

Electrocardiograms in school-aged healthy Polish children - an observational study.

BACKGROUND: Electrocardiographic (ECG) examination has long been used to assess cardiovascular function in clinical practice. Age-related ECG changes are observed as the cardiovascular system matures from the neonatal period to adolescence. AIM: This study aimed to evaluate effects of sex and age on ECG parameters in healthy schoolchildren. METHODS: The study included 336 healthy participants aged 5-12 years from the Masovian voivodeship. Children were divided into age groups of 5-8 and 9-12 years. Values for heart rate (HR), time intervals and amplitudes of P and QRS waves, and QRS axis for pediatric ECGs were estimated. RESULTS: Significant differences between boys and girls aged 5-8 years old were discovered for such parameters as PR interval, R-wave, S-wave, and the R/S ratio. Age-related decline in HR, Q-wave in V5 and V6, R-wave in V1-V4, and increase in QRS duration were noted. Girls presented a higher HR and shorter QRS than boys. HR, QRS axis, P wave amplitude in lead II, and amplitude of R and S in the precordial leads were different in our population than those previously reported. CONCLUSIONS: Pediatric ECG tracings were estimated for the first time for healthy Polish schoolchildren. Sex-related differences in selected ECG parameters in the younger age group were noticed. Several parameters differed from those previously reported in other ethnic populations. These findings are clinically significant and suggest that diagnostic criteria for pediatric ECG should be revised to establish if they are justifiable for the entire population.

Image quality and radiation doses of volumetric 320-row computed tomography angiography with prospective electrocardiogram-gating in the assessment of coronary arteries in neonates and infants.

BACKGROUND: Diagnostic imaging of coronary arteries is required in neonates and infants suspected of congenital or acquired coronary artery anomalies and in pre- and postoperative assessment of complex congenital heart diseases (CHD). AIM: Our study aimed to evaluate the image quality of volumetric 320-row computed tomography angiography (CTA) with prospective electrocardiogram (ECG)-gating for coronary arteries in neonates and infants with heart diseases, analyze factors influencing image quality and assess a radiation dose related to the procedure. METHODS: The study included 110 CTA performed in neonates and infants with CHD. RESULTS: CTA was performed in 37 girls and 73 boys at a median (interquartile range [IQR]) age of 3.0 (0.5-5.0) months, median (IQR) body weight of 5 (3.66-6.5) kg, and median heart rate (HR) of 133 (92-150) beats per minute. The orifices of the left coronary artery were visible in 100% of CTA, the orifices of the right coronary arteries were visible in 96%, whereas all coronary segments were assessable in 45% of CTA. Patients with non-diagnostic segments were significantly younger, median (IQR) age of 2.0 (0.21-5.00) months, had lower body weight of 4.6 (3.45-6.07) kg and faster HR of 136.5 (120-150) beats per minute (P <0.05) than patients with diagnostic image quality in all segments (4.0, 2-6 months, 6.0, 4.2-7 kg, and 130; 110-150 beats per minute, respectively; P <0.05). CONCLUSIONS: CTA performed with volumetric 320-row prospective ECG-gating allows for good visibility of the coronary arteries with an acceptable radiation dose. Children aged >15 days, with body weight >4.85 kg and HR <130 beats per minute are good candidates for excellent quality non-invasive CTA of all segments of coronary arteries.