Electrocardiograms are frequently obtained in infants and children. Training specific to paediatric electrocardiogram interpretation is necessary given that cardiac physiology and electrocardiogram findings in children are different than adults and change throughout infancy and childhood. Distributed practice may be an effective method to improve paediatric residency electrocardiogram education efforts.

A pre-survey was administered to paediatric and internal medicine/paediatrics residents to ascertain baseline comfort with electrocardiogram interpretation. Subsequently, residents were emailed a clinical vignette with an associated electrocardiogram and multiple-choice question 1–2 times monthly. After submitting their answer, residents were taken to a webpage explaining key concepts explored in the clinical vignette. After 6 and 12 months, a survey was administered asking residents to again rate their confidence in the same electrocardiogram interpretation skills.

The longitudinal email-based curriculum increased exposure to electrocardiogram training. Six months of participation in the curriculum correlated with significant increases in confidence in electrocardiogram interpretation skills. While there was no further increase in confidence seen at 12 months, the improvement seen at 6 months was durable. Participation in the curriculum most significantly correlated with changes in confidence in ability to utilise a stepwise approach for electrocardiogram interpretation.

The curriculum resulted in durable improvement in confidence in electrocardiogram interpretation skills for paediatric residents at our centre, suggesting that email-based distributive practice can be an effective method for skill and knowledge improvement for complex educational topics.

An important contributor to the decreased life expectancy of individuals with schizophrenia is sudden cardiac death. Arrhythmic disorders may play an important role herein, but the nature of the relationship between schizophrenia and arrhythmia is unclear.

To assess shared genetic liability and potential causal effects between schizophrenia and arrhythmic disorders and electrocardiogram (ECG) traits.

We leveraged summary-level data of large-scale genome-wide association studies of schizophrenia (53 386 cases, 77 258 controls), arrhythmic disorders (atrial fibrillation, 55 114 cases, 482 295 controls; Brugada syndrome, 2820 cases, 10 001 controls) and ECG traits (heart rate (variability), PR interval, QT interval, JT interval and QRS duration, n = 46 952–293 051). We examined shared genetic liability by assessing global and local genetic correlations and conducting functional annotation. Bidirectional causal relations between schizophrenia and arrhythmic disorders and ECG traits were explored using Mendelian randomisation.

There was no evidence for global genetic correlation, except between schizophrenia and Brugada syndrome (rg = 0.14, 95% CIs = 0.06–0.22, P = 4.0E−04). In contrast, strong positive and negative local correlations between schizophrenia and all cardiac traits were found across the genome. In the most strongly associated regions, genes related to immune and viral response mechanisms were overrepresented. Mendelian randomisation indicated that liability to schizophrenia causally increases Brugada syndrome risk (beta = 0.14, CIs = 0.03–0.25, P = 0.009) and heart rate during activity (beta = 0.25, CIs = 0.05–0.45, P = 0.015).

Despite little evidence for global genetic correlation, specific genomic regions and biological pathways emerged that are important for both schizophrenia and arrhythmia. The putative causal effect of liability to schizophrenia on Brugada syndrome warrants increased cardiac monitoring and early medical intervention in people with schizophrenia.

Reflex anoxic syncope is the result of an overreaction of the vagal system, resulting in hypotension and bradycardia or brief cardiac arrest. Because of the benign character and the absence of complications in short or long term, treatment is only necessary in case of frequent or severe clinical presentation. Treatment options are anticholinergic drugs or cardiac pacemaker placement. We investigated atropine treatment and aimed to examine if pacemaker placement can be avoided.

We retrospectively reviewed patients treated with atropine for severe reflex anoxic syncope in our centre from January 2017 until May 2023, and compared our results to those in the literature.

The study population consisted of 10 children, 70% female, with an age ranging from 5 months to 3 years (mean 14.5 months) when atropine treatment was started (dose 17–50 microg/kg/day). All patient’s parents reported adequate symptom management during atropine treatment, with complete resolution in 10%. Minor side effects were reported in 60% (dry mucosa in 40%, obstipation in 20%, and nausea or blurry vision in 10%).

We consider atropine a safe and effective treatment to manage reflex anoxic syncope with similar success rate to pacemaker implantation. However, pacemaker implantation entails substantial risk for complications (up to 25%) such as infection or technical problems and morbidity such as scar formation. This might be considered redundant for a benign and temporary condition, certainly given the possibility of other efficient treatment options. Consequently, we recommend atropine treatment over implantation of a cardiac pacemaker in children with severe reflex anoxic syncope.

Carvajal syndrome is a very rare autosomal recessive cardiocutaneous disorder caused by a desmosomal mutation in exon 24 of the desmoplakin gene. It manifests with woolly hair, epidermolytic palmoplantar keratoderma, and arrhythmogenic right ventricular cardiomyopathy. We herein present a patient with heart failure and dilated cardiomyopathy who was diagnosed with Carvajal syndrome because of dermatologic manifestations.

A seven-year-old girl was referred to our clinic due to decompensated heart failure and clinical deterioration. The patient had severe weakness, tachycardia, and tachypnea. She had a complaint of getting tired quickly for three weeks, and she had shortness of breath and abdominal pain for the last two days. She had hepatomegaly and woolly hair. Mild keratoderma was present on the soles of her feet. Echocardiography demonstrated biventricular dilatation, significantly impaired left ventricular systolic function (ejection fraction 22%), and moderate to severe mitral and tricuspid regurgitation. Molecular genetic evaluation was performed because of cutaneous and cardiac findings, which demonstrated a desmoplakin gene mutation. Homozygous mutation c.4297C > T (p.Gln1433*) was identified in desmoplakin gene, and the diagnosis of Carvajal syndrome was confirmed.

Syndromic types of arrhythmogenic right ventricular cardiomyopathy such as Carvajal syndrome are rare diseases. Awareness about cutaneous manifestations and genetic evaluation would help diagnosis and prevention of sudden death. Genetic counselling is needed in familial cases.

In pediatric multisystem inflammatory syndrome and isolated viral myocarditis/myopericarditis, autonomic nervous system function can be evaluated by a non-invasive method called heart rate variability. This study aims to evaluate heart rate variability in these two groups by comparing them with each other. This is the first study assessing these values in these two groups of patients.

Patients who are diagnosed with multisystem inflammatory syndrome in children and isolated viral myocarditis/myopericarditis at a university hospital from September 2021 to February 2023 are screened by electrocardiography, echocardiography, and 24-hour Holter monitoring. A healthy control group, compatible in age and gender with the patient groups, was selected from healthy subjects that applied to the hospital for palpitation, murmur, and/or chest pain. Heart rate variability parameters and related laboratory markers were analyzed and compared among the three groups.

There were 30 patients with multisystem inflammatory syndrome in children, 43 patients with isolated viral myocarditis/myopericarditis, and 109 participants in the healthy control group. Statistically significant differences were found in most of the heart rate variability parameters: standard deviation of normal to normal intervals (SDNN), the mean of the 5- minute RR interval standard deviations (SDNNİ), the standard deviation of 5-minute R wave to R wave(RR) interval means (SDANN), the root mean square of successive RR interval differences (RMSSD), and the percentage of the beats with a consecutive RR interval difference of more than 50 ms (pNN50%), very low frequency, high frequency, low frequency, triangular index, and low frequency/high-frequency ratio. Multisystem inflammatory syndrome in children patients had impaired and declined heart rate variability values compared to the other two groups. In patients with myocarditis/myopericarditis, we couldn’t find a significant difference in these parameters with the control group.

Heart rate variability can be used as an important non-invasive autonomic function parameter in determining prognosis and treatment plans, especially in patients diagnosed with multisystem inflammatory syndrome in children. This impairment of autonomic activity could be more prominent in patients with decreased left ventricular systolic functions.

Catecholaminergic polymorphic ventricular tachycardia is an ion channelopathy, caused by mutations in genes coding for calcium-handling proteins. It can coexist with left ventricular non-compaction. We aim to investigate the clinical and genetic characteristics of this co-phenotype.

Medical records of 24 patients diagnosed with catecholaminergic polymorphic ventricular tachycardia in two Chinese hospitals between September, 2005, and January, 2020, were retrospectively reviewed. We evaluated their clinical and genetic characteristics, including basic demographic data, electrocardiogram parameters, medications and survival during follow-up, and their gene mutations. We did structural analysis for a novel variant ryanodine receptor 2-E4005V.

The patients included 19 with catecholaminergic polymorphic ventricular tachycardia mono-phenotype and 5 catecholaminergic polymorphic ventricular tachycardia-left ventricular non-compaction overlap patients. The median age of onset symptoms was 9.0 (8.0,13.5) years. Most patients (91.7%) had cardiac symptoms, and 50% had a family history of syncope. Overlap patients had lower peak heart rate and threshold heart rate for ventricular tachycardia and ventricular premature beat during the exercise stress test (p < 0.05). Sudden cardiac death risk may be higher in overlap patients during follow-up. Gene sequencing revealed 1 novel ryanodine receptor 2 missense mutation E4005V and 1 mutation previously unreported in catecholaminergic polymorphic ventricular tachycardia, but no left ventricular non-compaction-causing mutations were observed. In-silico analysis showed the novel mutation E4005V broke down the interaction between two charged residues.

Catecholaminergic polymorphic ventricular tachycardia overlapping with left ventricular non-compaction may lead to ventricular premature beat/ventricular tachycardia during exercise stress test at lower threshold heart rate than catecholaminergic polymorphic ventricular tachycardia alone; it may also indicate a worse prognosis and requires strict follow-up. ryanodine receptor 2 mutations disrupted interactions between residues and may interfere the function of ryanodine receptor 2.