First Real-Life Data on the Diving Response in Healthy Children.

Swimming and diving are popular recreational activities, representing an effective option in maintaining and improving cardiovascular fitness in healthy people. To date, only little is known about the cardiovascular adaption to submersion in children. This study was conducted to improve an understanding thereof. We used a stepwise apnea protocol with apnea at rest, apnea with facial immersion, and at last apnea during whole body submersion. Continuous measurement of heart rate, oxygen saturation, and peripheral resistance index was done. Physiologic data and analysis of influencing factors on heart rate, oxygen saturation, and peripheral vascular tone response are reported. The current study presents the first data of physiologic diving response in children. Data showed that facial or whole body submersion leads to a major drop in heart rate, and increase of peripheral resistance, while the oxygen saturation seems to be unaffected by static apnea in most children, with apnea times of up to 75 s without change in oxygen saturation.

Use of the Apple Watch iECG in adult congenital heart disease patients.

INTRODUCTION: This study evaluates the accuracy of iECGs in comparison to the gold standard ECG in adult patients with congenital heart disease and recommends the appropriate iECG derivation based on the patient's characteristics. METHODS: In 106 adults (51 female, 55 male) with congenital heart disease, a gold standard 12-lead ECG was recorded, followed by three iECGs with the Apple Watch series 4, which correspond to Einthoven leads I, II, and III. Two experienced and independent cardiologists analyzed the time intervals, amplitudes, and polarities of the ECG parameters as well as the rhythm type and correlated the patient characteristics with the iECG parameters. RESULTS: The iECG parameters of all three iECG leads correlate strongly with those of the gold standard ECG, with exception of the P and T wave durations. We demonstrated that the informative value of the individual iECGs was independent of the patient's characteristics, in particular the heart axis, anatomy, and situs, even if the quality of the Einthoven III-like derivation was partially inadequate. The automatic rhythm analysis of the Apple Watch and the heart rhythm classification of a standard ECG analyzed manually by a cardiologist corresponded in 77%. CONCLUSION: iECG recordings of adults with congenital heart disease provide comparable results with Einthoven recordings I, II, and III of the 12-lead ECG and current data encourage the use of the Apple Watch not only in patients with structurally normal hearts but also in patients with congenital heart disease.

Accuracy of the Apple Watch single-lead ECG recordings in pre-term neonates.

INTRODUCTION: Telemedicine gained an increasing use throughout the last years. Lifestyle tools like the Apple watch seem to have an increasing spread even in remote areas and underdeveloped regions. The increasing availability of these tools offers the chance to use the health care functions of these devices to improve provision of professional medical care. First data on the use of the Apple Watch as a remote monitoring device in children have been reported, showing good acceptability and usability of the Apple Watch for symptom monitoring in children. This study aimed to evaluate the accuracy of the Apple Watch iECG in comparison to a standard 12-lead ECG in pre-term babies. METHODS: In this prospective, single-arm study, consecutive preterm neonates hospitalised in Leipzig University Hospital neonatal ICU were eligible. A 12-lead ECG and an iECG using Apple Watch 4 were performed. iECG and 12-lead ECG measurements were performed by a paediatric cardiologist. Cardiac rhythm was classified and amplitudes and timing intervals were analysed for comparability. RESULTS: Fifty preterm neonates, gestational week (23-36 weeks), and body weight (0.65-3.09 kg) were enrolled. Overall good quality and excellent correlation of the Apple Watch generated iECG in comparison to the standard 12-lead ECG could be demonstrated (p < 0.001). When interpreted by a paediatric cardiologist, a correct rhythm classification could be done in 100% of cases. CONCLUSION: The Apple Watch iECG seems to be a valuable tool to record an ECG comparable to lead I of the standard 12-lead ECG even in pre-term neonates. With a widespread availability and excellent connectivity, the Apple Watch iECG function may provide practitioners with a tool to send an iECG for interpretation to a paediatric cardiac specialist.

Evaluation of Clinical Course and Maintenance Drug Treatment of Supraventricular Tachycardia in Children During the First Years of Life. A Cohort Study from Eastern Germany.

Supraventricular tachycardia (SVT) is considered the most common cause of arrhythmia in children and infants. Regarding the likelihood of a spontaneous resolution of SVTs during the first years of life, drug treatment aims to bridge the time until children 'grow out' out of the arrhythmia. The choice of antiarrhythmic agents and the planning of maintenance therapy are mainly based on clinical experience and retrospective single- and multi-institutional analyses and databases from all over the world approaching differently to this topic. The current study aimed to evaluate the clinical course, pharmacological treatment strategies, and constellations of risk for recurrences in the management of SVTs in children aged 3 < years. The database of the Heart Center Leipzig, Department of Pediatric cardiology, was searched for pediatric patients aged < 3 years with a clinically documented SVT between 2000 and 2019 that received pharmacologic treatment. Patients with complex congenital heart disease or arrhythmias following cardiac surgery were excluded. 69 patients were included. Pharmacologic treatment, follow-up schedule, recurrences, outcomes, and risk factors for complicated courses are reported. Drug therapy of SVTs in young children remains a controversial topic with heterogeneous treatment and follow-up strategies applied. Risk factors for recurrences and/or stubborn clinical courses are difficult rhythm control with 3 or more antiarrhythmic drugs, ectopic atrial tachycardias, and a first occurrence of the SVT in the fetal period. Prospective studies are needed to sufficiently evaluate optimal treatment strategies.

Open field stress testing: finally an optimal method in young children? Reference values for mobile cardiopulmonary exercise testing in healthy children aged 4-8 years.

INTRODUCTION: Cardiopulmonary exercise testing represents the diagnostic tool for determining cardiopulmonary function. Especially in small children, exercise testing is extremely challenging. To address this problem, field testing has been implemented using small mobile devices. This study aims at using this protocol for developing normal values for cardiopulmonary exercise testing in very young children. MATERIAL AND METHODS: Healthy children aged 4-8 years were recruited. All children were tested according to an outdoor protocol, in which they were instructed to walk, then run slowly, then a little harder and at last run at full speed. Each step lasted for 2 minutes, except the last step, in which the children were instructed to maintain as long as possible. RESULTS: A total of 104 children (64 female/35 male, mean age 6.6 years) performed outdoor cardiopulmonary exercise testing using a mobile device. Almost all tests were completed successfully (95%). Despite a predominance of female study subjects, anthropometric values did not differ between boys and girls. V̇O2peak/kg, respiratory exchange ratio, VT1, heart rate at VT1, and time of exercise were also comparable between sexes. Generally, a tendency of higher maximal oxygen uptake could be observed in older children. CONCLUSION: Open field mobile cardiopulmonary exercise testing represents a novel approach in very young children. In this study, we were able to determine normal values of maximal oxygen uptake and OUES/kg for 4-8-year-old children. The method is easy to achieve and safe.

Performance of pacemaker leads in alternative lead positions after tricuspid valve replacement.

BACKGROUND: Bradycardic arrhythmias requiring pacemaker (PM) implantation are still common in patients in need of tricuspid valve replacement (TVR). Leaving an existing PM lead in an extravalvular position may represent a helpful alternative in special situations like the implantation of a mechanical TV. This study aimed to examine the short- to mid-term outcome of paravalvular leads concerning lead survival and prosthesis dysfunction in patients after TVR. METHODS: A retrospective case-control study of patients with TVR and ventricular pacing was conducted. Patients from the database of the Leipzig Heart Center were included. Data of the paravalvular lead group (PVG) and coronary sinus lead group (CSG) were compared to a control group with conventional transvalvular leads (TVG). RESULTS: Eighty patients with TVR and cardiac PM (TVG [n = 13], PVG [n = 40], and CSG [n = 27]) were included. The mean follow-up was 2.8 years. The rate of lead revisions (TVG 15.4%, PVG 2.5%, and CSG 7.5%) was lower in PVG but without significance (P = .286). The CSG demonstrated significantly higher pacing thresholds (1.4 V/0.8 ms) than TVG (0.5 V/0.4 ms), P = .004. However, the deterioration of threshold amplitudes during follow-up was similar in CSG (7.4%) and PVG (7.5%) compared with controls (7.7%). Function of TV prosthesis regarding development of stenosis or regurgitation showed a similarity between the groups (regurgitation PVG P = .692, CSG P = 1; stenosis PVG P = .586, CSG P = 0.69). CONCLUSION: Paravalvular positioning of PM leads seems to represent a reasonable alternative to the conventional transvalvular lead positioning concerning the lead and Tricuspid Valve prosthesis's function and durability in selected patients.

Junctional ectopic tachycardia in neonatal enterovirus myocarditis.

Differential diagnosis is challenging in poor conditioned neonates referred to the emergency room. Infectious disease is common, yet tachycardia should alert the clinician to look for cardiac arrhythmia and comprise. Tachycardia can lead to cardiomyopathy and should warrant further diagnostics for myocarditis, especially in rare or unusual combination of arrhythmias.

Quality of life and psychological co-morbidities in children and adolescents with cardiac pacemakers and implanted defibrillators: a cohort study in Eastern Germany.

INTRODUCTION: The implantation of a pacemaker or an implantable cardioverter-defibrillator during childhood may reduce quality of life and lead to mental health problems. This study aimed to evaluate potential mental health problems (i.e., depressive and anxiety symptoms) and quality of life in children with cardiac active devices in comparison to healthy peers. METHODS: We analysed data of children with pacemakers or implantable cardioverter-defibrillators aged 6-18 years. Quality of life, depressive and anxiety symptoms were assessed by standardised questionnaires. The results were compared to age-matched reference groups. RESULTS: Children with implantable cardioverter-defibrillator showed significant lower quality of life in comparison to reference group (p = 0.03), but there was no difference in quality of life between children with pacemaker and reference group. There was no significant difference in depressive symptoms between children with a cardiac rhythm device compared to reference group (self-report: p = 0.67; proxy report: p = 0.49). There was no significant difference in anxiety (p = 0.53) and depressive symptoms (p = 0.86) between children with pacemaker and children with implantable cardioverter-defibrillator. CONCLUSIONS: Living with an implantable cardioverter-defibrillator in childhood seems to decrease the patients' quality of life. Although children with pacemaker and implantable cardioverter-defibrillator don't seem to show more depressive and anxiety symptoms in comparison to their healthy peers, there still can be an increased risk for those children to develop mental health problems. Therefore, treating physicians should be aware of potential mental health problems and provide the patients and their families with appropriate therapeutic offers.

Different habitus but similar electrocardiogram: Cardiac repolarization parameters in children - Comparison of elite athletes to obese children.

INTRODUCTION: The standard 12-lead electrocardiogram (ECG) remains a widely used tool in the basic cardiac evaluation of children and adolescents. With the emergence of inherited arrhythmia syndromes, the period of cardiac repolarization has been the focus of attention. So far, data on cardiac repolarization and its normal variants in healthy children are scarce. This may cause uncertainties in the differentiation between pathologies and normal variants. As abnormal autonomic regulation seems to be a major influencing factor on cardiac repolarization, this study aimed to evaluate the parameters of cardiac repolarization of children in extremely good physical shape to obese children to improve knowledge about cardiac repolarization in these subgroups of pediatric patients that are vastly affected by the alterations of autonomic regulation. METHODS: A total of 426 pediatric volunteers (84 lean, healthy controls; 130 obese healthy pediatric volunteers; and 212 elite athletes) were enrolled in the study, and the parameters of cardiac repolarization were determined in 12-lead ECG. RESULTS: Most importantly, there were no pathological findings, neither in the healthy controls nor in the obese or athletes. Athletes showed overall shorter corrected QT intervals than children from the other groups. This is also true if a correction of the QT interval is performed using the Hodges formula to avoid bias due to a tendency to lower heart rates in athletes. Athletes showed the shortest Tpeak-to-end ratios between the groups. The comparison of athletes from primarily strength and power sports versus those from endurance sports showed endurance-trained athletes to have significantly longer QT intervals. CONCLUSIONS: This study suggests that neither obesity nor extensive sports seems to result in pathological cardiac repolarization parameters in healthy children. Therefore, pathology has to be assumed if abnormal repolarization parameters are seen and might not be simply attributed to the child's habitus or an excellent level of fitness.

Use of the Abbott EnSite Precision three-dimensional mapping system for the placement of an atrial pacemaker lead in a patient with congenital heart disease.

We report the use of the Abbott three-dimensional mapping system (EnSite Precision) for the placement of an atrial pacemaker lead in a patient after modified Fontan procedure. The mapping system was used for the identification of a promising pacing site in the complexly modified right atrium and to visualize the successful placement of an atrial lead at the same spot.

A novel technique for lead sparing tricuspid valve replacement in the case of a transvenous ICD lead.

Tricuspid valve (TV) surgery represents a complex consideration regarding lead management in patients with a transvenous ICD. The presented case shows favorable short-term results after lead sparing TV replacement, leaving an ICD lead in a paravalvular position. The described technique can be used in challenging cases of TV replacement.

Improved quality of life after treatment of prolonged asystole during breath holding spells with a cardiac pacemaker.

OBJECTIVES: To validate the physical and psychological effectiveness of cardiac pacing in pediatric patients with breath-holding spells (BHS) and prolonged asystole. MATERIALS AND METHODS: The records and clinical data of all the patients with BHS who presented to our center in the period of 2001-2013 were reviewed. All patients who received cardiac pacemaker implantation for prolonged asystole during BHS were included. In addition, the parents were asked to fill out a standardized quality of life (QOL) questionnaire. RESULTS: Seven patients were identified. The mean onset of symptoms was 7 month (1-12 months) of age, documented asystole was 12-21 seconds, and a permanent cardiac pacemaker device was implanted at a mean age of 23 months (8 months-3.9 years). No pacemaker related adverse events were recorded. Follow up showed immediate resolution from spells in four cases (4/7). Two patients (2/7) showed significant reduction of frequency and severity of spells, with complete elimination of loss of consciousness (LOC). One patient (1/7) with an additional neurologic disorder continued to have minor pallid BHS and eventually switched from pallid to cyanotic spells without further detection of bradycardia or asystole in holter examination. QOL questionnaire revealed significant reduction in subjective stress levels of patients (P = 0.012) and parents (P = 0.007) after pacemaker implantation. CONCLUSION: Cardiac pacing using appropriate pacemaker settings seems effective in the prevention of LOC and reduction of the frequency of BHS. Our results imply a reduction of subjective stress levels of patients and parents as well as an increased quality of everyday life. After all, randomized controlled trials of the influence of cardiac pacemaker implantation on subjective stress levels in patients with BHS are needed.

Performance of steroid eluting bipolar epicardial leads in pediatric and congenital heart disease patients: 15 years of single center experience.

OBJECTIVE: Cardiac pacing is sometimes required for patients with congenital heart disease for various reasons. Because of complex anatomy, repetitive previous heart surgery and patient size, epicardial leads are of special importance in these patients. Using epicardial leads has been discussed controversly regarding pacing performance and lead survival. The aim of this study was to review the long-term data on pacing performance as well as lead survival of epicardial leads implanted in our center. METHODS: Retrospective chart review and review of the literature. RESULTS: 82 consecutive pediatric patients or adult patients with congenital heart disease with 158 epicardial leads (Medtronic 4968, bipolar, steroid - eluting) were included. We found 1.2% pacemaker-related early postoperative complications. The incidence of lead dysfunction was 7.5% (12/158) for primary (i.e. directly related to the lead itself) lead dysfunction and 3.2% (5/158) of lead abandonment for reasons not directly related to the lead itself. Primary lead dysfunction occured after a median of 3.83 years. Lead survival at 2, 5 and 10 years was 98.7%, 93% and 92.4%. There were no infections reported. Stable median measurements for impedance (RA/RV/LV of 577/483/610 Ohm), sensing threshold (RA/RV/LV of 2.0/11.0/10.0 mV) and pacing threshold (RA/RV/LV of 0.75 V at 0.4 ms/1.0 V at 0.49 ms/1.0 V at 0.45 ms) indicated a good mid- to longterm performance. The only risk factor for primary lead dysfunction was young age at implantation. CONCLUSION: The use of epicardial leads in pediatric and adult patients with congenital heart disease shows good longterm outcomes in terms of pacing performance and lead survival. The authors encourage using epicardial leads in patients with congenital heart disease based on the patient's individual characteristics.

Ryanodine receptor mutations presenting as idiopathic ventricular fibrillation: a report on two novel familial compound mutations, c.6224T>C and c.13781A>G, with the clinical presentation of idiopathic ventricular fibrillation.

Idiopathic ventricular fibrillation (IVF) is a rare genetically determined disease causing unexpected cardiac death in otherwise healthy individuals. This study identified two novel, functional heterozygous mutations in the ryanodine receptor 2 (RyR2) gene in a family with IVF. In the presented case all the patients received a thorough diagnostic workup to exclude structural heart disease. Blood was drawn from the patients, and genetic testing was performed including amplification and sequencing of splice locations in two exons of the RyR2 gene. The mutations were detected in five symptomatic family members. The genetic status of the five affected family members remains unclear. No clinically affected patient is without mutation. At this writing, one family member with confirmed mutation is asymptomatic. The differentiation between catecholaminergic polymorphic ventricular tachycardia (CPVT) and IVF remains a difficult issue, mainly based on clinical characteristics and gross genetic classification. In our case, the family history, exercise testing, and epinephrine stress testing do not suggest an association of arrhythmia and adrenergic triggers, which makes CPVT rather unlikely despite the fact that genetic testing showed RyR2 mutations. Currently, knowledge concerning the functional meaning of genetic mutations is growing. Future exploration of these functional aspects might give further impetus to allocation of these patients to a specific diagnosis.

ECG phenomena: pseudopreexcitation and repolarization disturbances resembling ST-elevation myocardial infarction caused by an intraatrial rhabdomyoma in a newborn.

As is known from other reports, a rhabdomyoma or tumor metastasis may alter intracardiac electrical conduction, producing electrical phenomena like pseudopreexcitation or repolarization disturbances resembling ST-elevation myocardial infarction or Brugada's syndrome. We present a newborn with a giant atrial rhabdomyoma and additionally multiple ventricular rhabdomyomas. He presented with several electrocardiogram (ECG) phenomena due to tumor-caused atrial depolarization and repolarization disturbances. Except from the cardiac tumors, the physical status was within normal range. Initial ECG showed a rapid atrial tachycardia with a ventricular rate of 230 bpm, which was terminated by electrical cardioversion. Afterwards, the ECG showed atrial rhythm with frequent atrial premature contractions and deformation of the PR interval with large, broad P waves and loss of discret PR segment, imposing as pseudopreexcitation. The following QRS complex was normal, with seemingly abnormal ventricular repolarization resembeling ST-elevation myocardial infarction. The atrial tumor was resected with consequent vast atrial reconstruction using patch plastic. The ventricular tumors were left without manipulation. After surgery, pseudopreexcitation and repolarization abnormalities vanished entirely and an alternans between sinus rhythm and ectopic atrial rhythm was present. These phenomena were supposably caused by isolated atrial depolarization disturbances due to tumor-caused heterogenous endocardial activation. The seemingly abnormal ventricular repolarization is probably due to repolarization of the atrial mass, superimposed on the ventricular repolarization. Recognizably, the QRS complex before and after surgical resection of the rhabdomyoma is identical, underlining the atrial origin of the repolarization abnormalities before surgery.

Pharmacologic cardiac resynchronization of a 1-year-old boy with severe left ventricular dysfunction.

Postero-septal accessory pathways (AP) are a rare cause of intraventricular dyssynchrony and severe LV dysfunction in children. Beside the common treatment with radiofrequency ablation of septal substrates we present the case of a successful pharmacologic resynchronization in a 13/12 years old male toddler with Wolff-Parkinson-White syndrome (WPW) and severe LV dysfunction (left ventricular biplane EF of 31 %) due to intraventricular dyssynchrony with septal to posterior wall motion delay (SPWMD) of 350 ms. Interventricular mechanical delay (IVMD) was 65 ms. Using propafenone, pharmacologic cardiac resynchronization could be achieved. Pharmacologic resynchronization should be considered as safe and effective alternative to catheter ablation in very young children.

Dilated cardiomyopathy associated with dual-chamber pacing in infants: improvement through either left ventricular cardiac resynchronization or programming the pacemaker off allowing intrinsic normal conduction.

The etiology of dilated cardiomyopathy associated with congenital complete AV block has not yet been clarified. Two infants with AV block of autoimmune and surgical etiology, respectively, had received a dual-chamber right ventricular-based pacemaker and developed dilated cardiomyopathy with severe septal to left ventricular free-wall dyssynchrony 3.4 (0.9) years later. After 4 weeks of biventricular pacing and spontaneous junctional narrow QRS rhythm, respectively, both children showed significant improvement in left ventricular function along with reverse remodeling. Thus, electromechanical dyssynchrony associated with conventional right-ventricular-based DDD pacing may play a significant role in the development of dilated cardiomyopathy in the young.