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General clinicians and junior paediatricians may have little experience of interpreting paediatric electrocardiograms. Although the basic principles of cardiac conduction and depolarisation are the same as for adults, age related [...]

ObjectiveHypertrophic cardiomyopathy (HCM) is an important predictor of long-term outcomes in Friedreich’s ataxia (FA), but the clinical spectrum and survival in childhood is poorly described. This study aimed to describe the clinical characteristics of children with FA-HCM.Design and settingRetrospective, longitudinal cohort study of children with FA-HCM from the UK.Patients78 children (<18 years) with FA-HCM diagnosed over four decades.InterventionAnonymised retrospective demographic and clinical data were collected from baseline evaluation and follow-up.Main outcome measuresThe primary study end-point was all-cause mortality (sudden cardiac death, atrial arrhythmia-related death, heart failure-related death, non-cardiac death) or cardiac transplantation.ResultsThe mean age at diagnosis of FA-HCM was 10.9 (±3.1) years. Diagnosis was within 1 year of cardiac referral in 34 (65.0%) patients, but preceded the diagnosis of FA in 4 (5.3%). At baseline, 65 (90.3%) had concentric left ventricular hypertrophy and 6 (12.5%) had systolic impairment. Over a median follow-up of 5.1 years (IQR 2.4–7.3), 8 (10.5%) had documented supraventricular arrhythmias and 8 (10.5%) died (atrial arrhythmia-related n=2; heart failure-related n=1; non-cardiac n=2; or unknown cause n=3), but there were no sudden cardiac deaths. Freedom from death or transplantation at 10 years was 80.8% (95% CI 62.5 to 90.8).ConclusionsThis is the largest cohort of childhood FA-HCM reported to date and describes a high prevalence of atrial arrhythmias and impaired systolic function in childhood, suggesting early progression to end-stage disease. Overall mortality is similar to that reported in non-syndromic childhood HCM, but no patients died suddenly.

BackgroundVariants in the cardiac myosin-binding protein C gene (MYBPC3) are a common cause of hypertrophic cardiomyopathy (HCM) in adults and have been associated with late-onset disease, but there are limited data on their role in paediatric-onset HCM. The objective of this study was to describe natural history and clinical outcomes in a large cohort of children with HCM and pathogenic/likely pathogenic (P/LP) MYBPC3variants.Methods and resultsLongitudinal data from 62 consecutive patients diagnosed with HCM under 18 years of age and carrying at least one P/LP MYBPC3variant were collected from a single specialist referral centre. The primary patient outcome was a major adverse cardiac event (MACE). Median age at diagnosis was 10 (IQR: 2–14) years, with 12 patients (19.4%) diagnosed in infancy. Forty-seven (75%) were boy and 31 (50%) were probands. Median length of follow-up was 3.1 (IQR: 1.6–6.9) years. Nine patients (14.5%) experienced an MACE during follow-up and five (8%) died. Twenty patients (32.3%) had evidence of ventricular arrhythmia, including 6 patients (9.7%) presenting with out-of-hospital cardiac arrest. Five-year freedom from MACE for those with a single or two MYBPC3variants was 95.2% (95% CI: 78.6% to 98.5%) and 68.4% (95% CI: 40.6% to 88.9%), respectively (HR 4.65, 95% CI: 1.16 to 18.66, p=0.03).ConclusionsMYBPC3variants can cause childhood-onset disease, which is frequently associated with life-threatening ventricular arrhythmia. Clinical outcomes in this cohort vary substantially from aetiologically and genetically mixed paediatric HCM cohorts described previously, highlighting the importance of identifying specific genetic subtypes for clinical management of childhood HCM.

Biallelic hypomorphic variants in PPA2, encoding the mitochondrial inorganic pyrophosphatase 2 protein, have been recently identified in individuals presenting with sudden cardiac death, occasionally triggered by alcohol intake or a viral infection. Here we report 20 new families harboring PPA2variants.

AbstractThere is growing interest in the use of digital medicine to reduce the need for traditional outpatient follow-up. Remote interrogation of pacemakers and implantable cardioverter defibrillators is now possible with most devices. The aim of our study was to evaluate the safety and efficacy of virtual pacing clinics in following up children with pacemakers and implantable cardioverter defibrillators, including epicardial systems.Methods:The study was retrospective over 8 years (2010–2017), with review of patient records and analysis of downloads from the implantable cardiac devices to the virtual clinics.Results:A total of 75 patients were set up for virtual clinic follow-up during the study period, 94.5% with a pacemaker and 5.5% an implantable cardioverter defibrillator. The majority (76.8%) had an epicardial system. Data on lead impedance, battery longevity, programmed parameters, detected arrhythmias, percentage pacing and delivered defibrillator therapies were obtainable by download. Lead threshold measurements were obtainable via download in 83.7% of the devices, including epicardial systems. No concerning device issue was missed. In 15% of patients a major issue was detected remotely, including three patients with lead fractures. The virtual clinics resulted in fewer hospital attendances while enhancing monitoring and enabling more frequent device checks. The vast majority (91.4%) of families who responded to a questionnaire were satisfied with the virtual clinic follow-up.Conclusions:Virtual clinics allow safe and effective follow-up of children with pacemakers and implantable cardioverter defibrillators, including those with epicardial systems and are associated with high levels of parent satisfaction.

IMPORTANCE: Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM), but there is no validated algorithm to identify those at highest risk. OBJECTIVE: To develop and validate an SCD risk prediction model that provides individualized risk estimates. DESIGN, SETTING, AND PARTICIPANTS: A prognostic model was developed from a retrospective, multicenter, longitudinal cohort study of 1024 consecutively evaluated patients aged 16 years or younger with HCM. The study was conducted from January 1, 1970, to December 31, 2017. EXPOSURES: The model was developed using preselected predictor variables (unexplained syncope, maximal left-ventricular wall thickness, left atrial diameter, left-ventricular outflow tract gradient, and nonsustained ventricular tachycardia) identified from the literature and internally validated using bootstrapping. MAIN OUTCOMES AND MEASURES: A composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate implantable cardioverter defibrillator therapy, or sustained ventricular tachycardia associated with hemodynamic compromise). RESULTS: Of the 1024 patients included in the study, 699 were boys (68.3%); mean (interquartile range [IQR]) age was 11 (7-14) years. Over a median follow-up of 5.3 years (IQR, 2.6-8.3; total patient years, 5984), 89 patients (8.7%) died suddenly or had an equivalent event (annual event rate, 1.49; 95% CI, 1.15-1.92). The pediatric model was developed using preselected variables to predict the risk of SCD. The model’s ability to predict risk at 5 years was validated; the C statistic was 0.69 (95% CI, 0.66-0.72), and the calibration slope was 0.98 (95% CI, 0.59-1.38). For every 10 implantable cardioverter defibrillators implanted in patients with 6% or more of a 5-year SCD risk, 1 patient may potentially be saved from SCD at 5 years. CONCLUSIONS AND RELEVANCE: This new, validated risk stratification model for SCD in childhood HCM may provide individualized estimates of risk at 5 years using readily obtained clinical risk factors. External validation studies are required to demonstrate the accuracy of this model's predictions in diverse patient populations.

'The only difference between syncope and sudden death is that in syncope you wake up'--G L Engels [1] Syncope is a common problem in adolescence, with up to one in [...]

BackgroundThe AliveCor (Kardia) monitor attaches to a smartphone and allows a single-lead ECG to be recorded during symptoms. In 2016, we introduced the use of this smartphone device for investigating palpitations, without syncope, in children. The aim of our study was to review our experience with the smartphone device, comparing it with our previous standard conventional approach to cardiac event monitoring using the Cardiocall monitor, which uses skin electrodes and is given for a finite period.MethodsOver a period of 24 months, 80 smartphone monitors were issued and compared with the most recent 100 conventional event monitors. The number of ECG recordings received, arrhythmias documented, quality of ECG recordings and patient satisfaction were evaluated.ResultsMedian patient age was 11 years in the smartphone monitor group compared with 10 years in the conventional group. Seventy-nine of 80 (98%) patients with a smartphone monitor sent an ECG recorded during symptoms, compared with 62/100 (62%) from the conventional group. A total of 836 ECG recordings were sent from the smartphone monitors compared with 752 from the conventional group. Eight per cent of ECG recordings in each group were of inadequate quality for analysis. Twenty of 80 (25%) patients with a smartphone monitor had documented tachyarrhythmia compared with 6/100 (6%) patients with the conventional monitor (p<0.001). On comparison with the conventional approach, the smartphone monitor outperformed with respect to diagnostic yield and patient satisfaction.ConclusionsA smartphone-based event monitor allows simple, effective, long-term ECG event monitoring in children that is highly acceptable to the patient and parent.

AbstractBackgroundAutomated external defibrillators can be life-saving in out-of-hospital cardiac arrest.ObjectiveOur aim was to review our experience of prescribing automated external defibrillators for children at increased risk of sudden arrhythmic death.MethodsWe reviewed all automated external defibrillators issued by the Scottish Paediatric Cardiac Electrophysiology Service from 2005 to 2015. All parents were given resuscitation training according to the Paediatric Resuscitation Guidelines, including the use of the automated external defibrillator.ResultsA total of 36 automated external defibrillators were issued to 36 families for 44 children (27 male). The mean age at issue was 8.8 years. Diagnoses at issue included long QT syndrome (50%), broad complex tachycardia (14%), hypertrophic cardiomyopathy (11%), and catecholaminergic polymorphic ventricular tachycardia (9%). During the study period, the automated external defibrillator was used in four (9%) children, and in all four the automated external defibrillator correctly discriminated between a shockable rhythm – polymorphic ventricular tachycardia/ventricular fibrillation in three patients with one or more shocks delivered – and non-shockable rhythm – sinus rhythm in one patient. Of the three children, two of them who received one or more shocks for ventricular fibrillation/polymorphic ventricular tachycardia survived, but one died as a result of recurrent torsades de pointes. There were no other deaths.ConclusionParents can be taught to recognise cardiac arrest, apply resuscitation skills, and use an automated external defibrillator. Prescribing an automated external defibrillator should be considered for children at increased risk of sudden arrhythmic death, especially where the risk/benefit ratio of an implantable defibrillator is unclear or delay to defibrillator implantation is deemed necessary.

AbstractPrevious studies have identified that receiving specialist care close to home can positively influence patients’ experience. Despite this, a review of cardiology outpatient appointments at the Royal Hospital for Children in Glasgow demonstrated that a large number of families are bypassing their local children’s cardiology centre to attend cardiac clinics at the specialist children’s surgical centre. We used patient questionnaire, audit of local facilities, and examined the relationship between diagnosis and bypass numbers to better understand factors influencing this trend. Our results suggest that patient preference, short travelling distance to specialist children’s cardiac centre, a more severe cardiac diagnosis, and inconsistent local facilities, expertise, and support are likely to influence a family’s decision to bypass their local children’s cardiology centre.

Syncope in childhood is a common problem. In most children, syncope is benign, secondary to a disturbance in autonomic control of heart rate and blood pressure. It is increasingly evident that neurally mediated syncope is a heterogeneous group of conditions, necessitating a reclassification of autonomic disorders. New entities, such as postural orthostatic tachycardia and cerebral vasoconstrictive syncope, are recognized. The key to the diagnosis of syncope is a careful history. Tilt testing can be useful when the history is unclear. Unfortunately tilt test protocols vary, affecting specificity and sensitivity. The mainstay of therapy is reassurance. If symptoms are troublesome, Fludrocortisone and B-blockers remain the favored drugs. The efficacy of Midodrine and Serotonin Uptake Inhibitors is currently under review. Cardiac pacing is effective for those patients with severe episodes and demonstrated asystole. It is not known whether pacing would be effective for the majority who have neurally mediated syncope without significant bradycardia.

By capitalizing on the age detail available in interstate migration data, models have been designed to explain variation in migration streams as well as in areal variation in the volume or rate of migration. These models may be divided into three general classes: (1) gravity models; (2) intervening opportunities models; (3) aggregate ecological models incorporating "push-pull" mechanisms. This article reports efforts to analyze specific state-to-state flows of all older (55+) interstate migrants during the 1965-1970 period using a combination of these approaches.

The authors report a case of a 10-year-old boy who presented to the emergency department following an episode of syncope. While on telemetry, the child was found to have runs biventricular tachycardia. Catecholaminergic polymorphic ventricular tachycardia was diagnosed, and the case report discusses this rare but important diagnosis that should be considered in children presenting with syncope.

There have been few reports of the use of extracorporeal life support (ECLS) to provide cardiac support in children with low cardiac output as a result of arrhythmias. We present two patients in whom venoarterial ECLS was used to provide support for cardiogenic shock secondary to intractable supraventricular tachycardia. In both cases, the arrhythmia terminated once bypass was established. In one case, the time on ECLS was used to optimize drug treatment, and in the other case, radiofrequency ablation was successfully undertaken during ECLS.