Hypertrophic cardiomyopathy (HCM), a common cardiomyopathy in children, is an important cause of morbidity and mortality. Early recognition and appropriate management are important. An electrocardiogram (ECG) is often used as a screening tool in children to detect heart disease. The ECG patterns in children with HCM are not well described.ECGs collected from an international cohort of children, and adolescents (≤ 21 years) with HCM were reviewed. 482 ECGs met inclusion criteria. Age ranged from 1 day to 21 years, median 13 years. Of the 482 ECGs, 57 (12

The evolving breadth and complexity of the contemporary pediatric cardiology specialty requires regular, systematic analysis of the practice to ensure that training and certification requirements address the demands of real-world clinical experience. We report the process of the American Board of Pediatrics (ABP) for conducting such a practice analysis and revising the test content outline (TCO) for the pediatric cardiology subspecialty certification exam. A panel of 15 pediatric cardiologists conducted seven 2-h virtual meetings, during which they identified 37 unique tasks that represent the work a pediatric cardiologist may reasonably expect to perform within the first 5 years after training. These tasks were grouped into nine performance domains, similar to the entrustable professional activities (EPA), previously endorsed by the ABP in collaboration with the pediatric cardiology education community, and which represent the critical activities of the profession. The panel then enumerated the knowledge , skills , and abilities necessary to perform each task. These deliberations resulted in two work products: a practice analysis document (PAD) and subspecialty board TCO based on testable knowledge, skills, and abilities. Survey assessments of the panel’s work were then distributed to pediatric cardiology fellowship program directors and to practicing pediatric cardiologists for their input, which largely aligned with the panel’s recommendations. Survey responses were considered in the final revisions of the PAD and TCO. This approach to practice analysis proved to be an efficient process for describing the work performed by today’s pediatric cardiologists and the knowledge, skills, and abilities needed to competently perform that work.

Provide a brief summary of your article (100-150 words; no references or figures/tables). The synopsis appears only in the table of contents and is often used by indexing services such as PubMed. Genetic arrhythmia syndromes are rare, yet harbor the potential for highly consequential, often unpredictable arrhythmias or sudden death events. There has been historical uncertainty regarding the correct advice to offer to affected patients who are reasonably wanting to participate in sporting and athletic endeavors. In some cases, this had led to abundantly cautious disqualifications, depriving individuals from participation unnecessarily. Societal guidance and expert opinion has evolved significantly over the last decade or 2, along with our understanding of the genetics and natural history of these conditions, and the emphasis has switched toward shared decision making with respect to the decision to participate or not, with patients and families becoming better informed, and willing participants in the decision making process. This review aims to give a brief update of the salient issues for the busy physician concerning these syndromes and to provide a framework for approaching their management in the otherwise aspirational or keen sports participant.

Children and adolescents with congenital heart disease (CHD) have historically been restricted from vigorous exercise or competitive sports. With our growing understanding of the benefits of exercise and sports participation, we aim to review current recommendations for safe sports participation in children and adolescents with congenital heart disease. Recently published studies identify obesity to be highly prevalent in patients with CHD, especially as they age. This is more evident if the patients lead a sedentary lifestyle or are restricted from engaging in exercise. Recently published guidelines from American and European organizations offer disease-specific recommendations to allow for safe sports participation in children and adolescents with CHD. Sports restriction in children with CHD can have negative health and psychosocial consequences in an already vulnerable population. For patients who wish to participate in competitive sports, a comprehensive clinical evaluation is necessary to determine if participation is possible and to minimize risk associated with participation.

BACKGROUND Predictors of risk of lethal arrhythmic events (LAE) is poorly understood and may differ from adults in children with hypertrophic cardiomyopathy (HCM). OBJECTIVE The purpose of this study was to determine predictors of LAE in children with HCM. METHODS A retrospective data collection was performed on 446 children and teenagers 20 years and younger (290 [65%] male; mean age 10.1 +/- 5.7 years) with idiopathic HCM from 35 centers. Patients were classified as group 1 (HCM with LAE) if having a secondary prevention implantable cardioverter-defibrillator (ICD) or primary prevention ICD with appropriate interventions or group 2 (HCM without LAE) if having a primary prevention ICD without appropriate interventions. RESULTS There were 152 children (34%) in group 1 and 294 (66%) in group 2. Risk factors for group 1 by univariate analysis were septal thickness, posterior left ventricular (LV) wall thickness, lower LV outflow gradient, and Q wave > 3 mm in inferior electrocardiographic leads. Factors not associated with LAE were family history of SCD, abnormal blood pressure response to exercise, and ventricular tachycardia on ambulatory electrocardiographic monitoring. Risk factors for SCD by multivariate analysis were age at ICD placement (hazard ratio [HR] 0.9; P = .0025), LV posterior wall thickness z score (HR 1.02; P < .005), and LV outflow gradient < 30 mm Hg (HR 2.0; P < .006). LV posterior wall thickness z score >= 5 was associated with LAE. CONCLUSION Risk factors for LAE appear different in children compared to adults. Conventional adult risk factors were not significant in children. Further prospective studies are needed to improve risk stratification for LAE in children with HCM.

Obesity is associated with additional left ventricular hypertrophy (LVH) in adults with hypertrophic cardiomyopathy (HCM). It is not known whether obesity can lead to further LVH in children with HCM. Echocardiographic LV dimensions were determined in 504 children with HCM. Measurements of interventricular septal thickness (IVST) and posterior wall thickness (PWT), and patients’ weight and height were recorded. Obesity was defined as a body mass index (BMI) ≥ 99th percentile for age and sex. IVST data was available for 498 and PWT data for 484 patients. Patient age ranged from 2 to 20 years (mean ± SD, 12.5 ± 3.9) and 340 (68%) were males. Overall, patient BMI ranged from 7 to 50 (22.7 ± 6.1). Obesity (BMI 18–50, mean 29.1) was present in 140 children aged 2–19.6 (11.3 ± 4.1). The overall mean IVST was 20.5 ± 9.6 mm and the overall mean PWT was 11.0 ± 8.4 mm. The mean IVST in the obese patients was 21.6 ± 10.0 mm and mean PWT was 13.3 ± 14.7 mm. The mean IVST in the non-obese patients was 20.1 ± 9.5 mm and mean PWT was 10.4 ± 4.3 mm. Obesity was not significantly associated with IVST (p = 0.12), but was associated with increased PWT (0.0011). Obesity is associated with increased PWT but not IVST in children with HCM. Whether obesity and its impact on LVH influences clinical outcomes in children with HCM needs to be studied.

Aims Pathogenic gain-of-function variants in CACAN1C cause type-8 long QT syndrome (LQT8). We sought to describe the electrocardiographic features in LQT8 and utilize molecular modelling to gain mechanistic insights into its genetic culprits. Methods and results Rare variants in CACNA1C were identified from genetic testing laboratories. Treating physicians provided clinical information. Variant pathogenicity was independently assessed according to recent guidelines. Pathogenic (P) and likely pathogenic (LP) variants were mapped onto a 3D modelled structure of the Ca(v)1.2 protein. Nine P/LP variants, identified in 23 patients from 19 families with non-syndromic LQTS were identified. Six variants, found in 79% of families, clustered to a 4-residue section in the cytosolic II-III loop region which forms a region capable of binding STAC SH3 domains. Therefore, variants may affect binding of SH3-domain containing proteins. Arrhythmic events occurred in similar proportions of patients with II-III loop variants and with other P/LP variants (53% vs. 48%, P=0.41) despite shorter QTc intervals (47731ms vs. 515 +/- 37ms, P=0.03). A history of sudden death was reported only in families with II-III loop variants (60% vs. 0%, P=0.03). The predominant T-wave morphology was a late peaking T wave with a steep descending limb. Exercise testing demonstrated QTc prolongation on standing and at 4 min recovery after exercise. Conclusion The majority of P/LP variants in patients with CACNA1C-mediated LQT8 cluster in an SH3-binding domain of the cytosolic II-III loop. This represents a 'mutation hotspot' in LQT8. A late-peaking T wave with a steep descending limb and QT prolongation on exercise are commonly seen.

Pathogenic variants in the RYR2 gene are primarily associated with catecholaminergic polymorphic ventricular tachycardia (CPVT). Untreated CPVT is associated with a high risk of sudden cardiac arrest, which may be the first manifestation in an affected individual. The primary mechanism of disease for RYR2-associated CPVT is via gain-of-function missense variants, but at least one copy number variant (CNV) – an in-frame exon 3 deletion – has been associated with CPVT, arrhythmias, and other cardiomyopathies.

Introduction: Primary prevention (PP) ICD are increasingly placed in children with Hypertrophic Cardiomyopathy (HCM) to prevent SCD using adult criteria. The reliability of PPICD to prevent SCD is ...

Introduction: Children with Hypertrophic Cardiomyopathy (HCM) are at high risk for sudden cardiac death (SCD). Yet, risk stratification for SCD is poorly defined in children with HCM. Methods: We c...

•Entrustable professional activities define the tasks, knowledge and skills expected of a physician.•II The training guidelines define the curriculum and training environment.•Curricula components define knowledge, skills and scope of practice.

Introduction: Hypertrophic cardiomyopathy (HCM) is a genetic heart disease with diverse natural history. The hallmark of HCM is severe left ventricular hypertrophy (LVH). In adults with HCM, obesit...

Background and Purpose: This scientific statement provides an interprofessional, comprehensive review of evidence and recommendations for indications, duration, and implementation of continuous electro cardiographic monitoring of hospitalized patients. Since the original practice standards were published in 2004, new issues have emerged that need to be addressed: overuse of arrhythmia monitoring among a variety of patient populations, appropriate use of ischemia and QT-interval monitoring among select populations, alarm management, and documentation in electronic health records. Methods: Authors were commissioned by the American Heart Association and included experts from general cardiology, electrophysiology (adult and pediatric), and interventional cardiology, as well as a hospitalist and experts in alarm management. Strict adherence to the American Heart Association conflict of interest policy was maintained throughout the consensus process. Authors were assigned topics relevant to their areas of expertise, reviewed the literature with an emphasis on publications since the prior practice standards, and drafted recommendations on indications and duration for electrocardiographic monitoring in accordance with the American Heart Association Level of Evidence grading algorithm that was in place at the time of commissioning. Results: The comprehensive document is grouped into 5 sections: (1) Overview of Arrhythmia, Ischemia, and QTc Monitoring; (2) Recommendations for Indication and Duration of Electrocardiographic Monitoring presented by patient population; (3) Organizational Aspects: Alarm Management, Education of Staff, and Documentation; (4) Implementation of Practice Standards; and (5) Call for Research. Conclusions: Many of the recommendations are based on limited data, so authors conclude with specific questions for further research.

Significant mortality benefits have been documented in recipients of implantable cardioverter defibrillators (ICDs); however, the psychosocial distress created by the underlying arrhythmia and its potential treatments in patients and family members may be underappreciated by clinical care teams. The disentanglement of cardiac disease and device-related concerns is difficult. The majority of ICD patients and families successfully adjust to the ICD, but optimal care pathways may require additional psychosocial attention to all ICD patients and particularly those experiencing psychosocial distress. This state-of-the-science report was developed on the basis of an analysis and critique of existing science to (1) describe the psychological and quality-of-life outcomes after receipt of an ICD and describe related factors, such as patient characteristics; (2) describe the concerns and educational/informational needs of ICD patients and their family members; (3) outline the evidence that supports interventions for improving educational and psychological outcomes for ICD patients; (4) provide recommendations for clinical approaches for improving patient outcomes; and (5) identify priorities for future research in this area. The ultimate goal of this statement is to improve the precision of identification and care of psychosocial distress in ICD patients to maximize the derived benefit of the ICD.

INTRODUCTION We sought to determine the incidence, predictors, and consequences of new-onset atrial fibrillation (AF) following epicardial ventricular tachycardia (VT) ablation. METHODS AND RESULTS A total of 41 patients with no prior history of AF underwent epicardial VT ablation via a percutaneous subxiphoid approach. All patients were monitored continuously for 3 days following ablation and then via implantable cardiac defibrillator (ICD) or Holter monitoring. Mean age was 70.0 ± 11.3 years and mean ejection fraction was 30.3 ± 16.6%. In seven (17%) patients, the right ventricle (RV) was punctured during access with subsequent needle withdrawal without requiring surgical repair. Thirty patients (73%) were treated with amiodarone following ablation. Post-ablation, eight (19.5%) patients had documented new-onset AF within 7 days. All AF patients had clinical symptoms of pericarditis. One patient with AF was maintained on amiodarone post-procedure. Complications of AF included three patients who received inappropriate ICD shocks and one patient who developed a large, left atrial appendage clot. Acutely, all patients responded to short-term medical therapy or electrical cardioversion. At 18.0 ± 9.0 months of follow-up, no patient had recurrence of AF, and all were off antiarrhythmic drugs. One patient had typical atrial flutter requiring catheter ablation. Risk factors for AF included lack of amiodarone immediately after ablation (12.5 vs. 87.9%, P < 0.001), RV puncture (50.0 vs. 9.1%, P = 0.02), and epicardial ablation time >10 min (62.5 vs. 3.0%, P < 0.001). CONCLUSIONS Atrial fibrillation after epicardial ablation is common and can lead to ICD shocks and atrial thrombus formation. Short-term antiarrhythmic drug therapy and ICD reprogramming should be considered after epicardial VT ablation.

Concerns about the long-term effects of ionizing radiation for both patients and electrophysiology laboratory personnel combined with the development of electroanatomic mapping systems have made it possible to perform ablation of dysrhythmia substrates with minimal or no use of fluoroscopy.This article reviews the current literature demonstrating the safety and efficacy of this technique in pediatric patients.While most reports are retrospective and single-center studies, there is a growing interest in developing multicenter studies to further refine this technique.

BACKGROUND Nonsurgical subxiphoid pericardial access may be useful in ventricular tachycardia ablation and other electrophysiologic procedures but has a risk of right ventricular puncture.OBJECTIVE The purpose of this study was to identify a signature pressure frequency that would help identify the pericardial space and guide access.METHODS The study consisted of 20 patients (8 women and 12 men; mean age 59.1 +/- 14.2 years; left ventricular ejection fraction 25.2% +/- 12.2%; failed 1.8 +/- 0.5 endocardial ablations; unresponsive to 2.0 +/- 1.0 antiarrhythmic drugs; 6 ischemic cardiomyopathy, 12 nonischemic cardiomyopathy, 2 normal heart; 4 previous sternotomy) undergoing epicardial ventricular tachycardia ablation. After pericardial access was obtained, a 10Fr long sheath was used to record pressure inside the pericardium and pleural space. Pressures were analyzed using a fast Fourier transform to identify dominant frequencies in each chamber.RESULTS Mean pressures in the pleural space and the pericardium were not different (7.7 +/- 1.9 mmHg vs 7.8 +/- 0.9 mmHg, respectively). However, the pericardial space in each patient demonstrated two frequency peaks that correlated with heart rate (1.16 +/- 0.21 Hz) and respiratory rate (0.20 +/- 0.01 Hz), whereas the pleural space in each patient had a single peak correlating with respiratory rate (0.20 +/- 0.01 Hz).CONCLUSION The pericardial space demonstrates a signature pressure frequency that is significantly different from the surrounding space. This difference may make minimally invasive subxiphoid pericardial access safer for nonsurgeons and may have important implications for electrophysiologic procedures.

Background: Ablation of ventricular tachycardia (VT) reduces implantable cardioverter defibrillator shocks. Intracardiac ultrasound (ICE) can visualize and quantify the function of all left ventricular wall segments. We thus hypothesized that ICE could identify scar tissue and provide a guide to facilitate substrate‐guided VT ablation. Methods: Eighteen patients underwent VT ablation with real time ICE mapping from the right atrium and ventricle with online 3D‐image reconstruction of scar segments. The left ventricle was also scar mapped by traditional electroanatomic mapping (CARTO) for comparison. Images from these 2 scar mapping techniques were compared to each other as well as to a preprocedure transthoracic echocardiogram. Results: The average age was 65 ± 12 years and 12 (67%) were male (15 [83%] had ischemic cardiomyopathy). Two patients (12%) had recurrence of their clinical VT (1 remained on an antiarrhythmic medication, the other had a repeat ablation) over a follow‐up of 127 ± 33 days. No periprocedural or long‐term adverse events occurred. A total of 248 wall segments were analyzed. All 3 modalities were concordant in scar identification in 193 (78%) segments. The ICE segments correlated with the electroanatomic map in 213 (86%) segments versus 198 (80%), which correlated with transthoracic echocardiography and electroanatomic mapping (P = 0.046). Specifically, the ICE wall motion scores were closer to the electroanatomic mapping in the basal segments and showed a higher accuracy in ischemic heart disease. Conclusion: These data demonstrate that real time ICE images provide accurate chamber geometries and scar boundaries of the left ventricle. These scar borders were more accurate than transthoracic echocardiography and illustrate the feasibility of ICE for substrate‐based ablation for VT. (J Cardiovasc Electrophysiol, Vol. 21, pp. 678‐684, June 2010)

Background. Ventricular tachycardia (VT) ablation is an effective means to reduce ICD shocks. Intracardiac ultrasound can accurately visualize all left ventricular wall segments and quantify their ...