Despite its low prevalence, the potential diagnosis of catecholaminergic polymorphic ventricular tachycardia (CPVT) should be at the forefront of a paediatric cardiologists mind in children with syncope during exercise or emotions. Over the years, the number of children with a genetic diagnosis of CPVT due to a (likely) pathogenic RYR2 variant early in life and prior to the onset of symptoms has increased due to cascade screening programmes. Limited guidance for this group of patients is currently available. Therefore, we aimed to summarise currently available literature for asymptomatic patients with a (likely) pathogenic RYR2 variant, particularly the history of CPVT and its genetic architecture, the currently available diagnostic tests and their limitations, and the development of a CPVT phenotype – both electrocardiographically and symptomatic – of affected family members. Their risk of arrhythmic events is presumably low and a phenotype seems to develop in the first two decades of life. Future research should focus on this group in particular, to better understand the development of a phenotype over time, and therefore, to be able to better guide clinical management – including the frequency of diagnostic tests, the timing of the initiation of drug therapy, and lifestyle recommendations.

Sudden cardiac death is the most common mode of death during childhood and adolescence in hypertrophic cardiomyopathy, and identifying those individuals at highest risk is a major aspect of clinical care. The mainstay of preventative therapy is the implantable cardioverter-defibrillator, which has been shown to be effective at terminating malignant ventricular arrhythmias in children with hypertrophic cardiomyopathy but can be associated with substantial morbidity. Accurate identification of those children at highest risk who would benefit most from implantable cardioverter-defibrillator implantation while minimising the risk of complications is, therefore, essential. This position statement, on behalf of the Association for European Paediatric and Congenital Cardiology (AEPC), reviews the currently available data on established and proposed risk factors for sudden cardiac death in childhood-onset hypertrophic cardiomyopathy and current approaches for risk stratification in this population. It also provides guidance on identification of individuals at risk of sudden cardiac death and optimal management of implantable cardioverter-defibrillators in children and adolescents with hypertrophic cardiomyopathy.

Anomalous aortic origin of the left coronary artery from an incorrect aortic sinus has been reported as the second most common causes of sudden cardiac arrest in young athletes. Intramural course of the proximal left coronary artery is considered a high-risk morphology. It is associated with a slit-like ostium and elliptical shape of the proximal artery. In this case, all pre-operative cardiac images demonstrated a round ostium and round luminal shape of the left coronary artery, which suggested no evidence of intramural course. On intraoperative inspection, although the ostium and proximal left coronary artery appeared round, the patient had a long intramural course of the left coronary artery which our surgeons performed a successful unroofing procedure. The post-operative images showed a patent new ostium from the correct aortic sinus. These findings are very unusual and proved that we should not exclude intramural course even in the absence of a slit-like ostium and elliptical shape of the proximal left coronary artery.

In latent Wolff–Parkinson–White syndrome, ventricular pre-excitation is inapparent during sinus rhythm but carries the same possibility of sudden cardiac death and palpitations as overt Wolff–Parkinson–White syndrome. It is difficult to diagnose latent Wolff–Parkinson–White syndrome when a patient does not have syncope or palpitations. We report the case of an asymptomatic patient with latent Wolff–Parkinson–White syndrome detected on school heart screening using subtle electrocardiography findings.

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

An 11-year-old male was presented with exertional chest pain and was diagnosed with atresia of the left main coronary artery. A stress nuclear perfusion imaging was negative at initial presentation, and a vasodilator stress cardiac MRI was again negative 5 years later. The patient has fully participated in competitive sports for 6 years with no occurrence of cardiac events.

We present a case of sudden cardiac arrest in the field with complete neurological recovery in an 18-year-old athlete with phenotypic Noonan syndrome. Evaluation revealed interventricular septal thickness of 18 mm without left ventricular outflow tract obstruction and no other identifiable structural, electrophysiologic, or genetic abnormality except isolated heterozygous variant for desmoplakin DSP variant p.Lys2103Glu, with unknown clinical significance.

We present a young soldier presenting with aborted sudden cardiac death, who was found to have concomitant hypertrophic cardiomyopathy and Wolff–Parkinson–White syndrome. Along with pathological haemodynamic features of hypertrophic cardiomyopathy, an easily-inducible re-entrant tachycardia was clearly documented in our patient. Given the fatal potential of supraventricular tachycardia in hypertrophic cardiomyopathy, we postulated that his tachyarrhythmia could potentially trigger the event. Upon his refusal to receive implantable cardioverter/defibrillator therapy, we ablated anatomical arrhythmogenic substrate instead, and he remained uneventfully over 3 years on β-blocker.

Marine n-3 PUFA may improve autonomic dysfunction by an increase in heart rate variability (HRV) and may reduce the risk of malignant ventricular arrhythmias. Only a few smaller studies have examined such effects in patients on chronic dialysis, who often have autonomic dysfunction and a high risk of sudden cardiac death, which accounts for almost 30 % of all deaths. This cross-sectional study investigated the association between the plasma phospholipid content of n-3 PUFA and 24-h HRV or ventricular arrhythmias in patients on chronic dialysis. A 48-h Holter monitoring was performed on 169 patients on in-centre dialysis (83 %), home haemodialysis (10 %) or peritoneal dialysis (7 %) obtaining data on arrhythmias (n 152) and 24-h HRV (n 135). The mean overall HRV (standard deviation of normal intervals (SDNN)) was low and 71 % had a reduced overall HRV (SDNN<100 ms) indicating autonomic dysfunction. No significant associations between plasma phospholipid content of total marine n-3 PUFA, EPA (22 : 5n-3) or DHA (22 : 6n-3) and time-domain or frequency-domain HRV were detected in crude or adjusted linear regression analysis. However, a higher plasma phospholipid content of DHA was associated with a significantly lower proportion of patients with ventricular tachycardia (higher DHA-tertile: 9 % v. lower DHA-tertile: 28 %, P=0·02). In conclusion, the content of marine n-3 PUFA in plasma phospholipids was not associated with 24-h HRV, but a higher plasma phospholipid content of DHA was associated with a lower occurrence of ventricular tachycardia suggesting an antiarrhythmic effect of marine n-3 PUFA in patients on chronic dialysis.

Sudden death in young competitive athletes can be avoided by implementation of pre-participation screening programmes. A screening programme should be performed only by trained physicians and should include the athlete’s personal and family history, physical examination results, and the readings from a 12-lead-electrocardiogram. The athlete should undergo this screening programme every second year to detect progressive diseases. In addition, the programme should include detailed instructions to the athletes to pause training during infections in order to prevent sudden death due to myocarditis.

Military personnel generally under-consume n-3 fatty acids and overconsume n-6 fatty acids. In a placebo-controlled, double-blinded study, we investigated whether a diet suitable for implementation in military dining facilities and civilian cafeterias could benefit n-3/n-6 fatty acid status of consumers. Three volunteer groups were provided different diets for 10 weeks. Control (CON) participants consumed meals from the US Military’s Standard Garrison Dining Facility Menu. Experimental, moderate (EXP-Mod) and experimental-high (EXP-High) participants consumed the same meals, but high n-6 fatty acid and low n-3 fatty acid containing chicken, egg, oils and food ingredients were replaced with products having less n-6 fatty acids and more n-3 fatty acids. The EXP-High participants also consumed smoothies containing 1000 mg n-3 fatty acids per serving, whereas other participants received placebo smoothies. Plasma and erythrocyte EPA and DHA in CON group remained unchanged throughout, whereas EPA, DHA and Omega-3 Index increased in EXP-Mod and EXP-High groups, and were higher than in CON group after 5 weeks. After 10 weeks, Omega-3 Index in EXP-High group had increased further. No participants exhibited changes in fasting plasma TAG, total cholesterol, LDL, HDL, mood or emotional reactivity. Replacing high linoleic acid (LA) containing foods in dining facility menus with similar high oleic acid/low LA and high n-3 fatty acid foods can improve n-6/n-3 blood fatty acid status after 5 weeks. The diets were well accepted and suitable for implementation in group feeding settings like military dining facilities and civilian cafeterias.

It has largely been accepted that pre-participation screening for student athletes is necessary, but there is still no consensus on the most effective and efficient ways to accomplish this. Most clinical strategies are based on retrospective case series. By applying the European Society of Cardiology and Seattle criteria, electrocardiography appears to afford the lowest false-positive rate for identifying potentially dangerous cardiac abnormalities in athletes. Prospective, randomised trials may help determine the most effective primary prevention. Normative data for age, gender, and ethnicity for screening tools need to be formulated to further reduce false-positive results. Targeted advanced screening aimed at the highest risk groups may be the most beneficial and cost-effective application of primary prevention.

Improved public awareness and advances in medical diagnostics have resulted in the development of criteria to determine eligibility or disqualification for the athlete with cardiovascular abnormalities. Simultaneously, protocols have been developed for athletes with concussion or orthopaedic injuries to guide team physicians and consultants in return-to-play decisions. However, there are currently inadequate data to allow the development of such protocols for athletes with cardiovascular abnormalities who have undergone treatment. Further complicating the decision process is the designation of the team physician as the ultimate authority in making return-to-play decisions – where the team physician often is an employee of the team and supports the team’s goal and players as well as the individual athlete. This review will discuss the ethical dilemma of the team physician and the role of the cardiovascular consultant. Following this, current data and practices regarding return to play will be discussed for the following conditions or diagnoses: following catheter ablation for supraventricular tachycardia; following pacemaker or implantable cardioverter-defibrillator implantation; unexplained syncope; and the athlete with a genetic mutation in the absence of any phenotype of associated disease. These recommendations will undoubtedly continue to evolve and improve and should be considered at this time as a point of departure.

A young woman with Epstein’s anomaly had a large endocardial scar in the atrialised ventricular myocardium. In patients with significant preoperative ventricular scarring, more information is needed to help appropriately manage the risk of ventricular arrhythmias and sudden death.

The recreational use of illicit drugs remains an enormous and growing problem throughout the United States of America and around the world. Cocaine is most frequently thought of when considering the cardiovascular toxicity of illicit drugs. The association of cocaine use with sudden death due to myocardial ischaemia and infarction is well recognised, and this risk appears to be amplified by concomitant cigarette smoking and alcohol consumption. Like cocaine, amphetamine and its derivatives lead to indirect stimulation of the autonomic nervous system through the release of norepinephrine, dopamine, and serotonin in nerve terminals of the central and autonomic nervous systems. However, amphetamine lacks the ion channel-blocking properties of cocaine. Also similar to cocaine, coronary artery spasm may be induced in individuals with or without atherosclerotic disease and may lead to myocardial infarction. With the movement across the United States of America to legalise marijuana, or cannabis, for medicinal and recreational purposes, it is important to consider its potential deleterious effects. Marijuana has long been thought to have very few adverse effects with the exception of long-term dependence. There are, however, scattered reports of acute adverse events up to and including sudden death. These appear to be due to myocardial infarction. In conclusion, the incidence of sudden death associated with the use of these drugs varies from rare in the case of marijuana use to not infrequent with some drugs such as cocaine. It is important for care providers to recognise the potential for drug abuse when caring for a sudden cardiac arrest survivor.