The Case for Home AED in Children, Adolescents, and Young Adults Not Meeting Criteria for ICD.

Children, adolescents, and young adults with conditions such as cardiomyopathies and channelopathies are at higher risk of sudden cardiac death caused by lethal arrhythmias, especially ventricular fibrillation. Timely defibrillation saves lives. Patients thought to be at significantly high risk of sudden death typically undergo placement of an implantable cardioverter-defibrillator. Patients thought to be at lower risk are typically followed medically but do not undergo implantable cardioverter-defibrillator placement. However, low risk does not equal no risk. Compared with the general population, many of these patients are at significantly higher risk for lethal arrhythmias. We make the case that such individuals and families will benefit from having an at-home automatic external defibrillator. Used in conjunction with conventional measures such as training on cardiopulmonary resuscitation, an at-home automatic external defibrillator could lead to significantly shortened time to defibrillation with better overall and neurological survival. We recommend that the cost of such home automatic external defibrillators should be covered by medical insurance.

mRNA Coronavirus Disease 2019 Vaccine-Associated Myopericarditis in Adolescents: A Survey Study.

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions.

Health Care Policy and Congenital Heart Disease: 2020 Focus on Our 2030 Future.

The congenital heart care community faces a myriad of public health issues that act as barriers toward optimum patient outcomes. In this article, we attempt to define advocacy and policy initiatives meant to spotlight and potentially address these challenges. Issues are organized into the following 3 key facets of our community: patient population, health care delivery, and workforce. We discuss the social determinants of health and health care disparities that affect patients in the community that require the attention of policy makers. Furthermore, we highlight the many needs of the growing adults with congenital heart disease and those with comorbidities, highlighting concerns regarding the inequities in access to cardiac care and the need for multidisciplinary care. We also recognize the problems of transparency in outcomes reporting and the promising application of telehealth. Finally, we highlight the training of providers, measures of productivity, diversity in the workforce, and the importance of patient-family centered organizations in advocating for patients. Although all of these issues remain relevant to many subspecialties in medicine, this article attempts to illustrate the unique needs of this population and highlight ways in which to work together to address important opportunities for change in the cardiac care community and beyond. This article provides a framework for policy and advocacy efforts for the next decade.

Sudden Death in the Young: Information for the Primary Care Provider.

There are multiple conditions that can make children prone to having a sudden cardiac arrest (SCA) or sudden cardiac death (SCD). Efforts have been made by multiple organizations to screen children for cardiac conditions, but the emphasis has been on screening before athletic competition. This article is an update of the previous American Academy of Pediatrics policy statement of 2012 that addresses prevention of SCA and SCD. This update includes a comprehensive review of conditions that should prompt more attention and cardiology evaluation. The role of the primary care provider is of paramount importance in the evaluation of children, particularly as they enter middle school or junior high. There is discussion about whether screening should find any cardiac condition or just those that are associated with SCA and SCD. This update reviews the 4 main screening questions that are recommended, not just for athletes, but for all children. There is also discussion about how to handle post-SCA and SCD situations as well as discussion about genetic testing. It is the goal of this policy statement update to provide the primary care provider more assistance in how to screen for life-threatening conditions, regardless of athletic status.

CPR and AEDs save lives: insuring CPR--AED education and CPR--AED access in schools.

PURPOSE OF REVIEW: Sudden cardiac arrest and sudden cardiac death are less common in children and adolescents than in the adult population. The outcomes from sudden cardiac arrest are generally quite poor in all ages and some data suggest that they are worse in the child and adolescent age group. In addition, the incidence of bystander cardiopulmonary resuscitation (CPR) and automated external defibrillator (AED) use is generally quite low although it is somewhat variable across communities. This review has been written in order to review the data for pediatric bystander CPR and AED use as it relates to out-of-hospital cardiac arrest (OHCA) survival. The purpose of this article is also to review endeavors at CPR--AED education in the context of improving both community bystander CPR/AED interventions and OHCA survival. Finally, this review will attempt to suggest some potential educational interventions in order to increase both bystander CPR-AED use and OHCA survival in local communities. RECENT FINDINGS: Findings from several recent studies suggest that the incidence of bystander CPR--AED intervention is relatively low and that OHCA survival is also quite low in most communities. Several studies also suggest that community educational efforts can increase bystander CPR--AED interventions. The increase in OHCA survival may be attributed to the community educational efforts, yet that increase in survival is relatively small. A prospective high-school study has shown that in this very specific environment, a high incidence of CPR--AED use can be achieved, and that as a result the OHCA survival can be relatively high. SUMMARY: It would be beneficial to attempt to ensure that all schools have a CPR--AED program along with a group of individuals trained to do CPR and use an AED. Not only should all schools have a cardiac emergency response plan (CERP) but all schools should have CPR--AED programs and all students should learn CPR and AED use prior to graduation. This strategy will ensure that we will have a community of individuals who would perform CPR and use an AED in the community and that in so doing we could increase the incidence of bystander CPR/AED use and increase the OHCA survival rate.

Screening for prevention of sudden death in the young: what is new?

PURPOSE OF REVIEW: The optimal approach to screening young people to decrease the risk of sudden death remains unknown. It deserves the passionate attention that researchers, clinicians and families have given it. The new data from January 2018 to July 2019 are reviewed here. RECENT FINDINGS: Cardiac findings associated with a risk of sudden death were reported in 0.4% of screened athletes. Well run programs continue report varying sensitivity for screening ECGs (between 86 and 100%). One major article reported a higher incidence of sudden death in young people than has been previously published (6.8/100 000 athletes). SUMMARY: The rate of important findings in sophisticated screening programs is approximately 0.4%, suggesting that this is near the population rate of detectable disease in most athletic groups. ECGs are unquestionably capable of detecting disease that can be missed by history and physical, but the performance characteristics of ECGs continue to vary from study to study. In addition, the underlying cost and infrastructure of ECG and echocardiographic screening remains unaddressed by the recent literature. A few small studies have started to look at alternative technology approaches to ECG screening. VIDEO ABSTRACT.

Safety of Sports for Young Patients With Implantable Cardioverter-Defibrillators: Long-Term Results of the Multinational ICD Sports Registry.

BACKGROUND: Despite safety concerns, many young patients with implantable cardioverter-defibrillators (ICDs) participate in sports. We undertook a prospective, multinational registry to determine the incidence of serious adverse events because of sports participation. The primary end points were death or resuscitated arrest during sports or injury during sports because of arrhythmia or shock. Secondary end points included system malfunction and incidence of ventricular arrhythmias requiring multiple shocks for termination. METHODS: Athletes with ICDs aged ≤21 years were included in this post hoc subanalysis of the ICD Sports Registry. Data on sports and clinical outcomes were obtained by phone interview and medical records review. ICD shocks and clinical details of lead malfunction were classified by 2 electrophysiologists. RESULTS: A total of 129 young athletes participating in competitive (n=117) or dangerous (n=12) sports were enrolled. The mean age was 16 years (range, 10-21; 40% female; 92% white). The most common diagnoses were long QT syndrome (n=49), hypertrophic cardiomyopathy (n=30), and congenital heart disease (n=16). The most common sports were basketball and soccer, including 79 varsity/junior varsity high school and college athletes. During a median follow-up of 42 months, 35 athletes (27%) received 38 shocks. There were no occurrences of death, arrest, or injury related to arrhythmia, during sports. There was 1 ventricular tachycardia/ventricular fibrillation storm during competition. Freedom from lead malfunction was 92.3% at 5 years and 79.6% at 10 years. CONCLUSIONS: Although shocks related to competition/practice are not uncommon, there were no serious adverse sequelae. Lead malfunction rates were similar to previously reported in unselected pediatric ICD populations. CLINICAL TRIAL REGISTRATION: URL: https://www.clinicaltrials.gov . Unique identifier: NCT00637754.

The Second Annual Think Tank on Prevention of Sudden Cardiac Death in the Young: Developing a rational, reliable, and sustainable national health care resource. A report from the Cardiac Safety Research Consortium.

Sudden cardiac death in the young (SCDY) spans gender, race, ethnicity, and socioeconomic class. The loss of any pediatric patient is a matter of national and international public health concern, and focused efforts should be aimed at preventing these burdensome tragedies. Prepared by members of the Cardiac Safety Research Consortium, this White Paper summarizes and reports the dialogue at the second Think Tank related to the issues and the proposed solutions for the development of a national resource for screening and prevention of SCDY. This Think Tank, sponsored by the Cardiac Safety Research Consortium and the United States Food and Drug Administration, convened on February 18, 2016, in Miami, FL, to identify and resolve the barriers that prevent early identification of patients at risk for SCDY. All potential stakeholders including national and international experts from industry, medicine, academics, engineering, and community advocacy leaders had an opportunity to share ideas and collaborate.

2017 American Heart Association Focused Update on Pediatric Basic Life Support and Cardiopulmonary Resuscitation Quality: An Update to the American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.

This focused update to the American Heart Association guidelines for cardiopulmonary resuscitation (CPR) and emergency cardiovascular care follows the Pediatric Task Force of the International Liaison Committee on Resuscitation evidence review. It aligns with the International Liaison Committee on Resuscitation's continuous evidence review process, and updates are published when the International Liaison Committee on Resuscitation completes a literature review based on new science. This update provides the evidence review and treatment recommendation for chest compression-only CPR versus CPR using chest compressions with rescue breaths for children <18 years of age. Four large database studies were available for review, including 2 published after the "2015 American Heart Association Guidelines Update for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care." Two demonstrated worse 30-day outcomes with chest compression-only CPR for children 1 through 18 years of age, whereas 2 studies documented no difference between chest compression-only CPR and CPR using chest compressions with rescue breaths. When the results were analyzed for infants <1 year of age, CPR using chest compressions with rescue breaths was better than no CPR but was no different from chest compression-only CPR in 1 study, whereas another study observed no differences among chest compression-only CPR, CPR using chest compressions with rescue breaths, and no CPR. CPR using chest compressions with rescue breaths should be provided for infants and children in cardiac arrest. If bystanders are unwilling or unable to deliver rescue breaths, we recommend that rescuers provide chest compressions for infants and children.

The Care of Children With Congenital Heart Disease in Their Primary Medical Home.

Congenital heart disease (CHD) is the most common birth anomaly. With advances in repair and palliation of these complex lesions, more and more patients are surviving and are discharged from the hospital to return to their families. Patients with CHD have complex health care needs that often must be provided for or coordinated for by the primary care provider (PCP) and medical home. This policy statement aims to provide the PCP with general guidelines for the care of the child with congenital heart defects and outlines anticipated problems, serving as a repository of current knowledge in a practical, readily accessible format. A timeline approach is used, emphasizing the role of the PCP and medical home in the management of patients with CHD in their various life stages.

The Pulmonary Circulation in the Single Ventricle Patient.

In recent decades, survival of children with complex congenital heart disease has improved considerably. Specifically, children with a variety of congenital heart defects resulting in 'single ventricle' physiology can now undergo palliative surgery that allows survival beyond the neonatal period, and in many cases into adulthood, despite having a single functional ventricular pumping chamber supplying both the pulmonary and systemic circulation. Our growing understanding of the functionally univentricular heart has resulted in freedom from Fontan failure of >50% at 25 years post-Fontan. Yet there is still a fair amount of knowledge to be gained, specifically as it relates to the pulmonary circulation in this group of patients. Knowledge gaps relate not only to the pulmonary circulation after Fontan operation, but also at each stage of the single ventricle surgical palliation, including the native physiology prior to any intervention. The pulmonary circulation is affected by multiple issues related to the single ventricle, including specific details of the anatomy unique to each patient, any intervention(s) undertaken, and potential complications such as aortopulmonary collaterals, protein losing enteropathy, plastic bronchitis, venovenous collaterals, pulmonary arteriovenous fistulae, ventricular dysfunction, pulmonary venous stenosis, and more. This chapter will review the current knowledge with regard to the pulmonary circulation in the single ventricle patient, primarily after the Fontan operation. Additionally, it is our hope to help the practitioner assess the pulmonary circulation in the single ventricle patient; we will also discuss the evidence behind and approach to treatment strategies in order to optimize the pulmonary circulation in this complex group of patients.

Prevention of sudden cardiac death in the young: Developing a rational, reliable, and sustainable national health care resource. A report from the Cardiac Safety Research Consortium.

This White Paper, prepared by members of the Cardiac Safety Research Consortium, discusses important issues regarding sudden cardiac death in the young (SCDY), a problem that does not discriminate by gender, race, ethnicity, education, socioeconomic level, or athletic status. The occurrence of SCDY has devastating impact on families and communities. Sudden cardiac death in the young is a matter of national and international public health, and its prevention has generated deep interest from multiple stakeholders, including families who have lost children, advocacy groups, academicians, regulators, and the medical industry. To promote scientific and clinical discussion of SCDY prevention and to germinate future initiatives to move this field forward, a Cardiac Safety Research Consortium-sponsored Think Tank was held on February 21, 2015 at the US Food and Drug Administration's White Oak facilities, Silver Spring, MD. The ultimate goal of the Think Tank was to spark initiatives that lead to the development of a rational, reliable, and sustainable national health care resource focused on SCDY prevention. This article provides a detailed summary of discussions at the Think Tank and descriptions of related multistakeholder initiatives now underway: it does not represent regulatory guidance.

How to develop and execute a public health agenda: From grass roots to legislation.

Recent experiences with lay public cardiopulmonary resuscitation and use of ambulatory external defibrillators have been demonstrated to improve survival from out-of-hospital cardiac arrests from an abysmal 10% or less to as high as 30%. Though rare, sudden cardiac arrest in presumably healthy young people have been highly publicised over the last 25 years and have motivated the institution of school- and community-based programmes that facilitate first-responder resuscitation, including defibrillation. These efforts often begin at a grass roots level (usually parent-inspired advocacy groups) and through collaborative activities involving the schools, local businesses, and local administrative agencies are now having a meaningful impact in selected communities. The current focus of these activities is on school- and sports venue-based ambulatory external defibrillator programmes and formal cardiopulmonary resuscitation education in high schools. The extension of these programmes to the level of state mandates has been slower, and even when enacted, public funding has usually not been approved.

Risk of Ventricular Arrhythmias and Association with Ondansetron.

OBJECTIVES: To evaluate the use of ondansetron in a tertiary care pediatric health system, assess the incidence of ventricular tachyarrhythmia within 24 hours of ondansetron, and identify the characteristics of children experiencing a ventricular tachyarrhythmia after ondansetron, to identify potential risk factors. STUDY DESIGN: This retrospective chart review identified children ≤18 years of age who received ondansetron within 24 hours prior to a ventricular tachyarrhythmia. Those identified were evaluated for other diagnoses, concomitant medication use, electrolyte abnormalities, or underlying conduction abnormalities that may have contributed to the arrhythmia. RESULTS: A total of 199 773 doses of ondansetron were administered to 37 794 patients over 58 009 visits. Average dose was 0.13 mg/kg/dose (range 0.005-0.86 mg/kg/dose). Seven patients received ondansetron within 24 hours prior to a ventricular arrhythmia. All 7 patients had underlying congenital cardiac conduction abnormalities (n = 3) or other major cardiac diagnoses (n = 4). In clinical review, torsades de pointes was found in only 1 of the 7 patients. CONCLUSIONS: This retrospective study found the risk of ventricular arrhythmia within 24 hours after ondansetron administration was 3 in 100 000 patients treated annually (0.003%). Children with major cardiac conditions could be considered for electrocardiogram screening and continuous cardiac monitoring while receiving ondansetron. Our findings do not support recommendations for electrocardiogram screening or continuous monitoring of other pediatric populations receiving ondansetron.

Cardiac Emergency Response Planning for Schools: A Policy Statement.

A sudden cardiac arrest in school or at a school event is potentially devastating to families and communities. An appropriate response to such an event-as promoted by developing, implementing, and practicing a cardiac emergency response plan (CERP)-can increase survival rates. Understanding that a trained lay-responder team within the school can make a difference in the crucial minutes between the time when the victim collapses and when emergency medical services arrive empowers school staff and can save lives. In 2015, the American Heart Association convened a group of stakeholders to develop tools to assist schools in developing CERPs. This article reviews the critical components of a CERP and a CERP team, the factors that should be taken into account when implementing the CERP, and recommendations for policy makers to support CERPs in schools.

Attention deficit hyperactivity disorder medications in children with heart disease.

PURPOSE OF REVIEW: Attention deficit hyperactivity disorder (ADHD) is quite common in the general pediatric population, Its incidence is thought to be even higher in the population of patients with congenital heart disease, especially in those patients with complex disease and who have had cardiac surgical interventions early in life. There has been controversy as to the safety of ADHD medications, especially in the latter population of patients. This compendium is meant to review the effects of the ADHD medications and the safety of these medications in patients with either known or undiagnosed congenital heart disease. RECENT FINDINGS: The concern with regard to the use of ADHD medications has been as a result of the reports of sudden unexpected deaths among patients taking stimulant drugs for ADHD. Therefore, the question of whether or not stimulant drugs increase the risk of adverse cardiovascular events has led to a discussion of the appropriate use of these drugs in patients with known cardiovascular disease, as well as a discussion as to the appropriate evaluation in order to identify undiagnosed 'at-risk' patients with congenital heart disease or arrhythmias. This article will review and amplify these discussions, as well as the conclusions that have come forth as a result of these discussions. SUMMARY: Currently available data suggest that there is no evidence for serious adverse cardiovascular complications in children with known cardiovascular diseases including patients of congenital heart disease who are treated with stimulant medications. Despite this, if the patient does have known cardiac disease, or if the history and physical examination is suggestive of cardiac disease, it is suggested that consultation/evaluation with a pediatric cardiologist occur. It is extremely unlikely that stimulant medications would be contraindicated in almost any condition that falls under this category. However, a few specific cardiac conditions might tailor the choice of the specific ADHD medication. Therefore the coordination of care between the primary care physician, the ADHD medication-prescribing physician, and the pediatric cardiologist may be very important in this circumstance.

Ventricular Assist Device in Single-Ventricle Heart Disease and a Superior Cavopulmonary Anastomosis.

Our objective is to describe the use of a ventricular assist device (VAD) in single-ventricle patients with circulatory failure following superior cavopulmonary anastomosis (SCPA). We performed a retrospective chart review of all single-ventricle patients supported with a VAD following SCPA. Implantation techniques, physiologic parameters while supported, medical and surgical interventions postimplant, and outcomes were reviewed. Four patients were supported with an EXCOR Pediatric (Berlin Heart Inc., The Woodlands, TX, USA) following SCPA for a median duration of 10.5 days (range 9-312 days). Selective excision of trabeculae and chords facilitated apical cannulation in all patients without inflow obstruction. There were two pump exchanges in the one patient supported for 312 days. Two patients were evaluated by cardiac catheterization while supported. Three of four patients were successfully bridged to transplantation. One patient died while supported. All patients had significant bleeding at the time of transplantation, and one required posttransplant extracorporeal membrane oxygenation with subsequent full recovery. VAD support can provide a successful bridge to transplantation in patients with single-ventricle circulation following SCPA. A thorough understanding of the challenges encountered during this support is necessary for successful outcomes.