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From the American Academy of PediatricsPolicy Statement

Pediatric Sudden Cardiac Arrest

SECTION ON CARDIOLOGY AND CARDIAC SURGERY
Pediatrics April 2012, 129 (4) e1094-e1102; DOI: https://doi.org/10.1542/peds.2012-0144
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Abstract

Pediatric sudden cardiac arrest (SCA), which can cause sudden cardiac death if not treated within minutes, has a profound effect on everyone: children, parents, family members, communities, and health care providers. Preventing the tragedy of pediatric SCA, defined as the abrupt and unexpected loss of heart function, remains a concern to all. The goal of this statement is to increase the knowledge of pediatricians (including primary care providers and specialists) of the incidence of pediatric SCA, the spectrum of causes of pediatric SCA, disease-specific presentations, the role of patient and family screening, the rapidly evolving role of genetic testing, and finally, important aspects of secondary SCA prevention. This statement is not intended to address sudden infant death syndrome or sudden unexplained death syndrome, nor will specific treatment of individual cardiac conditions be discussed. This statement has been endorsed by the American College of Cardiology, the American Heart Association, and the Heart Rhythm Society.

KEY WORDS
  • Abbreviations:
    AAP —
    American Academy of Pediatrics
    AED —
    automated external defibrillator
    AHA —
    American Heart Association
    CPR —
    cardiopulmonary resuscitation
    CPVT —
    catecholaminergic polymorphic ventricular tachycardia
    ECG —
    electrocardiography
    EMS —
    emergency medical services
    HCM —
    hypertrophic cardiomyopathy
    LQTS —
    long QT syndrome
    PPE —
    preparticipation evaluation
    SCA —
    sudden cardiac arrest
    SIDS —
    sudden infant death syndrome
    VF —
    ventricular fibrillation

    • Incidence of Pediatric Sudden Cardiac Arrest

    In the United States, there is no centralized or mandatory registry for pediatric sudden cardiac arrest (SCA). Available data generally are collected through media reports, from lay SCA advocacy groups, or from peer-reviewed publications, often from major referral medical centers. Episodes of resuscitated cardiac arrest (aborted cardiac death) are even more difficult to document retrospectively. The Centers for Disease Control and Prevention has estimated that every year in the United States, approximately 2000 patients younger than 25 years will die of SCA.1 Other older reports estimate the frequency of SCA in children and adolescents to be between 0.8 and 6.2 per 100 000 per year.2–6 Two studies suggest that the frequency of SCA in adolescents and young adults actually may be increasing.7,8 Although SCA occurs even at young ages and at rest, the likelihood of child and young adult SCA for those with underlying cardiovascular disease is increased by athletic participation.9 Nonetheless, 2 studies from Maron et al10,11 estimate fewer than 100 cases of SCA in young US competitive athletes each year. An Italian study reported a baseline incidence of SCA in young competitive athletes at 1:25 000 before implementing a national screening program.12 Corrado et al identified a 2.5 times relative risk for SCA attributable to sports activity in adolescent and young adult athletes versus an age-matched nonathletic population,13 related to underlying cardiac disorders.

    Reporting and referral biases affect our knowledge of SCA incidence. The difficulty in determining cause of death in patients with primary cardiac electrical disorders (so-called “autopsy negative”) must be acknowledged. Many of these now recognized electrical disorders have been described only recently, confounding older literature that details the cause of pediatric SCA identified at autopsy.

    Cardiac Disorders Predisposing Youth to SCA

    Underlying cardiac disorders associated with pediatric and young adult SCA are listed in Table 1. In general, causes can be considered (1) structural or functional (expected to be identified with echocardiography or at autopsy); (2) primary electrical (most commonly associated with structurally and functionally normal hearts); or (3) other, including use of illicit drugs and stimulants (eg, cocaine, ephedra) or prescription medications (eg, erythromycin, ketoconazole, carbamazepine). The reader is directed to reference texts and previous publications for more detail about each of these individual conditions.14,15

    View this table:
    TABLE 1

    Cardiac Disorders Predisposing to Pediatric and Young Adult SCA

    Genetics of Pediatric SCA

    The identification of disease-causing genetic mutations is progressing rapidly in all areas of medicine. Evaluation of large cohorts of ostensibly healthy individuals has begun to catalog the common polymorphisms and the background rate of rare genetic variants of uncertain significance within the general population. For cardiac disease, the science of genotypic evaluation has not yet advanced to the point at which genotype alone (isolated from clinical phenotypic description) can routinely and accurately risk stratify for clinical outcome. Many cardiac disorders, including hypertrophic cardiomyopathy (HCM) and the cardiac ion channelopathies, are known to be genetic.16,17 Several studies have documented the efficacy of genetic testing of first-degree relatives of persons who have died of SCA. A 2003 study18 reported cardiac symptoms in 27% of surviving relatives, with a 22% incidence of unexpected premature sudden death in addition to the proband in any relative and a 6% incidence of sudden death in a first-degree relative. After evaluating 49 cases of young autopsy-negative SCA, Tester and Ackerman19 reported 17 cases with genetic/molecular evidence for long QT syndrome (LQTS) or catecholaminergic polymorphic ventricular tachycardia (CPVT) disease–causing mutations; 9 (53%) of these cases had a family history of SCA or syncope documented by the medical examiner. A personal history of syncope, seizure, or previous cardiac arrest was detailed for 7 individuals whose deaths were attributable to SCA. In a 2005 report, genetic testing established a likely cause of death in 17 of 43 autopsy-negative persons (40%). Genetic testing of family members revealed an additional 151 presymptomatic and undiagnosed disease carriers (average of 8.9 per family).20

    Recognizing the genetic nature of many of the disorders listed in Table 1, the role of a detailed, comprehensive family history (and considering consultation with an expert in cardiac genetics) is readily apparent. The primary goal is prospective identification of any family member, even if asymptomatic, who is genotypically or phenotypically affected by a disease entity predisposing a person to SCA. A 2008 publication discusses the role of family history for evaluating cardiomyopathy and ion channelopathies predisposing people to SCA.21 A 3-generation pedigree as a family history tool is highly effective for clinical evaluation; a family history template suggested by the US Surgeon General’s Family History Initiative is available free at www.hhs.gov/familyhistory.

    Warning Signs and Symptoms

    Although SCA may be the sentinel event, symptoms in patients with structural-functional or primary electrical disorders may, in fact, be relatively common before SCA. Often, these warning signs or symptoms may be misinterpreted or disregarded by both family members and medical personnel. These points were emphasized in a 1996 publication22 that summarized 9 previous studies. Preceding symptoms of dizziness, chest pain, syncope, palpitations, or dyspnea and a family history of premature, unexpected sudden death were noted in 25% to 61% of the study population. Deaths were exertion-related in 8% to 33% of cases. A study of 162 young persons (15–34 years of age)23 undergoing autopsy evaluation after SCA found 92 cases with a history of syncope/presyncope, chest pain, palpitations, or dyspnea; 26 of these subjects had a family history of SCA. In a study of natural death in people 5 to 35 years of age,24 the most common cause of sudden death was presumed arrhythmia in those with no or minimal heart disease (29%). Eleven percent of cases were exercise-associated. A history of SCA was reported in 4.5% of first-degree relatives of the descendents. Importantly, symptoms may be nonspecific and confusing in athletes, who may overexert until physical exhaustion.

    In most cases, the immediate cause of SCA is a lethal ventricular tachyarrhythmia (ventricular fibrillation [VF] or pulseless ventricular tachycardia) causing cardiac collapse. Some of these arrhythmias (eg, torsades de pointes, the characteristic tachyarrhythmia associated with LQTS) may be short lived and self-terminating, causing episodes of syncope/presyncope or episodes of seizure-like activity.19,22–25 These neurologic signs and symptoms may direct referral to a neurologist, inadvertently misdirecting the patient away from cardiac evaluation and, thus, delaying correct diagnosis and treatment. These tachyarrhythmia-associated SCA events must be distinguished from the well-recognized but poorly understood entity called sudden unexpected death in epilepsy.26,27 In the latter, this primary neurologic event may cause a cardiac death, mediated through abnormalities of cardiovascular autonomic function.28 Chest pain is almost never present in patients with primary electrical disorders but is more likely in patients with cardiomyopathies,29,30 congenital coronary artery abnormalities,31 or aortic disease (eg, dissection or rupture associated with Marfan syndrome32). Other nontypical cardiac presentations also may misdirect patients to other consulting medical subspecialties. Symptoms suggestive of exercise-induced bronchospasm may be present in patients with HCM and dilated or restrictive cardiomyopathy. Cardiomyopathy-associated wheezing is attributable to decreased left ventricular compliance, mitral insufficiency, or pulmonary venous hypertension with pulmonary edema. Failure of empirical exercise-induced bronchospasm medication or normal pulmonary function testing should prompt cardiovascular evaluation. Drowning or near-drowning has been associated with LQTS and CPVT.33,34 Approximately 5% to 10% of sudden infant death syndrome (SIDS) cases may stem from channelopathic mutations in genes associated with LQTS, Brugada syndrome, and CPVT.35–38 Congenital deafness has been noted in some types of LQTS.39 Patients with congenital deafness should be evaluated for LQTS if the deafness is not otherwise associated with another recognized syndrome or anomaly. Febrile seizures may be a presenting sign of children affected with Brugada syndrome.40

    Screening Techniques

    The role of any screening effort is to identify individuals at risk; unaffected or low-risk individuals should be cleared, and conversely, those affected should be appropriately restricted counseled, and treated. Not all SCAs can be foreseen, even in the best of circumstances. No screening protocol has yet proven to be effective in this role or validated as highly effective.

    Sports Preparticipation Evaluation and Cardiovascular Risk Assessment

    As noted by aforementioned studies, it is estimated that as many as half of pediatric SCA cases exhibited a personal/familial sudden death warning sign or symptom (such as previous exercise-triggered faint or family history of premature unexplained sudden death). Thus, there is an opportunity to identify individuals at risk for pediatric SCA without technology-based screening programs, such as the 12-lead electrocardiography (ECG) and echocardiography; however, despite the aforementioned data supporting the fact that preceding warning signs and symptoms may be present in many patients and families at risk for SCA, most published studies have not substantiated the efficacy of current athletic preparticipation evaluation (PPE) processes. Only 3% of 158 athletes with SCA were suspected of having cardiovascular disease using a PPE screen, leading the authors of a 1996 study to conclude that “pre-participation screening appeared to be of limited value for identification of underlying cardiovascular abnormalities.”41 The 1996 study was retrospective, and the details of the PPE questionnaire used and the adequacy of PPE were not reported. This report also predated description of some of the disease entities now known to cause pediatric SCA. More recently, an investigation from the United Kingdom concluded that family history and personal symptom questionnaire alone were inadequate for identification of at-risk patients and families.42 The 2008 UK study used a comprehensive PPE format and trained examiners, with little reported benefit, which reveals the potential failure of a single PPE at 1 point in time.

    In contrast to a single PPE initiated only before athletic participation, a more thorough cardiovascular risk-assessment process, applied throughout childhood and adolescence (the continuum of well-child care), can be provided for any patient, of any age, by any care provider (Table 2). Patient and family histories can and do change over time, necessitating an update of information for the care provider. Families should be encouraged to provide complete and accurate responses concerning history. PPE screening of athletes exclusively omits >25 million US schoolchildren who do not participate in sports. Postponing cardiovascular risk assessment until a more formal high school or collegiate athletic PPE screen also will delay detection of patients and families at risk for SCA before high school. For any PPE or cardiovascular risk assessment to succeed, medical providers must be aware of sudden death warning signs and symptoms and respond appropriately with comprehensive cardiovascular evaluation, referral, treatment, and activity restrictions as appropriate. The use of competent and qualified examiners is still a necessity, but recent data reveal that 35% of states allow nonphysicians with little cardiovascular training to perform the screening evaluations.43

    View this table:
    TABLE 2

    Pediatric Sudden Cardiac Death Risk Assessment Form

    View this table:

    Disclosures

    The American Heart Association (AHA) has documented a 12-element recommendation for preparticipation screening of competitive athletes.44 Another PPE, sponsored and endorsed by the American Academy of Pediatrics (AAP) and 5 other agencies, is now widely used throughout the United States for childhood and adolescent athletic PPE.45 This document acknowledges the wide variation in physician competencies and documentation for PPE examinations. Many states have endorsed the use of this PPE to eliminate unnecessary variability and to more effectively screen for cardiovascular risk. The updated fourth edition of this PPE form became available in April 2010.

    None of the aforementioned PPEs or cardiovascular risk-assessment tools have been validated but serve as standard templates for more comprehensive screening. Likewise, no true sensitivity or specificity data exist for prediction of risk of SCA by PPE. Among the many warning signs and symptoms, the following 4 appear to represent more ominous positive responses (based on expert opinion):

    1. Have you ever fainted, passed out, or had a seizure suddenly and without warning, especially during exercise or in response to auditory triggers such as doorbells, alarm clocks, and ringing telephones?

    2. Have you ever had exercise-induced chest pain or shortness of breath?

    3. Are you related to anyone with sudden, unexplained, and unexpected death before the age of 50?

    4. Are you related to anyone who has been diagnosed with a sudden death–predisposing heart condition such as HCM, LQTS, Brugada syndrome, and so forth? (See Table 1.)

    Once a cardiovascular disorder listed on Table 1 is suspected or diagnosed, referral to and management by pediatric/adult cardiologists or heart rhythm specialists experienced with the particular sudden death–predisposing heart condition is crucial.

    Another important time, resource, and cost-benefit issue centers around obtaining the detailed and accurate cardiovascular risk assessment or PPE forms in the primary care office setting. This time-consuming process is currently poorly reimbursed and difficult to prioritize and validate in a busy practice.

    ECG Screening

    Although some data suggest that SCA screening may be enhanced with the addition of ECG, broad-scale ECG screening has not been tested or implemented in the United States. Mandatory screening of Japanese schoolchildren since 197346,47 has demonstrated a greater sensitivity of ECG versus history and physical examination. Competitive Italian athletes undergo required PPE and ECG, with ECG reportedly demonstrating 77% greater power to detect HCM than history and physical examination alone.2 Italy also has reported a newborn ECG screening program to identify infants at risk for SIDS secondary to abnormal cardiac repolarization.48 For Olympic athletes, the International Olympic Medical Committee issued a screening protocol including ECG in 2004.49 A 2005 European Society of Cardiology consensus statement on cardiovascular preparticipation screening of young competitive athletes recommends 12-lead ECG in addition to focused history and physical examination.50 Some US studies have suggested that ECG screening may be cost-effective on the basis of estimated cost per year of lives saved.51,52

    The 2007 AHA scientific statement/screening guidelines44 (coauthored by S.B. and M.J.A.) did not recommend standard ECG assessment, however, citing false-positive and false-negative results, cost-effectiveness, feasibility, and medicolegal concerns. Wide-scale ECG screening would require a major infrastructure enhancement not currently available in the United States. Recent reassessment of ECG “normal” values has helped to decrease false-positive findings.53 Competitive athletes are known to have unusual but occasionally benign ECG findings, consistent with “athlete’s heart,” that must be differentiated from ECG findings attributable to pathologic conditions.54

    The role of routine ECG screening in the United States to prevent SCA is not settled and will require more data and debate. Readers are referred to recently published debates of the subject for further details.55,56

    Molecular Autopsy

    The genetic nature of many cardiac ion channelopathies predisposing youth to pediatric SCA is being defined rapidly.17 When children die suddenly, there may be no previous evaluation or diagnosis. Conventional autopsies often fail to identify a condition responsible for sudden death. These autopsy-negative cardiac conditions have previously defied complete definition. As already described, complete evaluation of a child who died of SCA through detailed clinical and targeted genetic testing of immediate family members may identify specifically the cause of SCA and direct appropriate care and genetic counseling to surviving family members. The cardiac channel postmortem genetic analysis (also known as “molecular autopsy”)57 remains a research test but soon may evolve into a standard clinical practice. Unfortunately, current standards of care for autopsy do not yet ensure that a postmortem sample suitable for DNA analysis is retained. Further, despite the evidence that approximately 25% to 35% of autopsy-negative sudden unexplained death is channelopathic, health insurance companies currently do not accept responsibility for molecular autopsy of the deceased in the United States. The cost would befall the medical examiner and, ultimately, the community; however, far more expensive testing of all first-degree surviving family members currently is used clinically and reimbursed. An important next step will be the development of guidelines at a public health level for postmortem genetic testing.

    Primary Prevention of SCA

    Primary prevention of SCA depends on patient diagnosis, specific etiology, and etiology-specific treatment. Treatment options include but are not limited to medical therapy, device therapy (eg, pacemakers, internal cardioverter defibrillators), activity-restriction guidelines, avoidance of certain classes of medications, and family emergency preparedness. The details of primary prevention, given that they are etiology specific and prescribed by a consulting cardiologist, are beyond the scope of this policy statement.

    Secondary Prevention of SCA

    When SCA primary prevention strategies (ie, patient identification, treatment, activity restriction, and counseling) have failed, SCA still may occur, and secondary prevention (resuscitative) efforts are required. The AHA has proposed a “chain-of-survival”58 beginning with early symptom recognition and 911 emergency medical services (EMS) contact, followed by effective bystander cardiopulmonary resuscitation (CPR), early defibrillation, and finally, advanced hospital care. The published outcomes for out-of-hospital pediatric cardiac arrest are dismal; survival to hospital discharge occurs in approximately <10% of children, and many have severe neurologic sequelae.59–63 Poor outcomes may be related to prolonged periods of no cardiac output, in part because many out-of-hospital arrests are unwitnessed, and only approximately 30% of children received bystander CPR61 (note also that bystander CPR more than doubles patient survival rates64).

    Bystanders report that they do not perform CPR because of panic or fear of failure65 and unwillingness to perform mouth-to-mouth rescue breathing. Recent studies suggest that “compression-only” CPR may be more effective than standard CPR with ventilation,66,67 by using faster (approximately 100 per minute) and deeper compressions, in adults for witnessed nonasphyxial arrest (arrest not secondary to, for example, drowning, hanging, or carbon monoxide poisoning). To date, there are no pediatric studies with respect to compression-only CPR. Because pediatric patients are more likely to experience respiratory arrests, compression only may not be suitable. Two studies report VF as the initial rhythm in 19% to 24% of out-of-hospital pediatric cardiac arrests, excluding deaths attributable to SIDS.68,69 VF and ventricular tachycardia generally have been considered more favorable initial SCA rhythms than either asystole or pulseless electrical activity, with a higher rate of survival to hospital discharge, when prompt defibrillation (termination of VF) and return of an organized perfusing rhythm is achieved. As part of the chain of survival, public access defibrillation using automated external defibrillators (AEDs) has a prominent role.70 Data from witnessed VF arrest in adults show that appropriate use of AEDs can lead to long-term survival rates >70%.71,72 AEDs have now been recommended for children younger than 8 years,73,74 with still insufficient scientific evidence to warrant official recommendation for or against AED use in children aged 1 year or younger. A 2007 AAP policy statement addressed current pathophysiology of VF and recommendations for AED use in children; readers are referred to this publication for further detail.75

    School AED Programs

    The average school-aged child spends 28% of the day and 14% of his or her total annual hours in school.76 In addition, adults (parents, grandparents, teachers, staff, and visitors) crowd our schools. As an area of higher traffic, schools have become sites for implementation of AED programs. In 1 report, 67% of schools activate EMS for an emergency involving a student, and 37% activate EMS for an emergency involving an adult.76 A 2007 report detailed a 16-year history of SCA in Seattle city and King county schools, providing a framework for reasonable and rational school-based emergency programs.77

    A growing number of states have mandated school AED programs. The cost-effectiveness of school AED programs has been reported by Berger et al.78 Key components for a comprehensive school-preparedness program include education and all-staff awareness, knowledge and application of effective bystander CPR techniques, implementation of a lay-rescuer AED program, and written emergency action plans,79 with all steps reinforced with effective communication throughout the school campus and periodic practice drills. Current principles guiding this recommendation for schools, primary clinicians, and school physicians have been detailed in the AAP policy statement “Medical Emergencies Occurring at School.”80 At this time, there are no published data to support the efficacy of home AEDs.81

    Recommendations for Pediatric Care Providers

    Evidence-based recommendations frequently are designated as class I, II, or III, indicating the supporting level of evidence. For pediatric SCA, the level of evidence does not permit a meaningful use of this terminology. This statement has been endorsed by the American College of Cardiology, the American Heart Association, and the Heart Rhythm Society.

    All steps in the primary and secondary SCA-prevention strategies should be optimized if pediatric SCA is to be prevented.

    Recommendations

    Important steps for consideration include:

    1. Recognize the warning signs and symptoms of SCA, including those that may “misdirect” initial evaluation to noncardiac specialties and, thus, delay correct diagnosis.

    2. Understand the role of comprehensive and accurate family history and pedigree for preventing SCA stemming from inherited cardiac genetic disorders.

    3. Use standardized PPE forms and processes to minimize unnecessary variation.

    4. Ensure that identified patients and/or families with known or suspected cardiac disorders are referred to a pediatric cardiac center for further comprehensive evaluation and management. Appropriate secondary testing may include ECG, echocardiography, exercise testing, or genetic testing, as indicated.

    5. Advocate for autopsy evaluation by a medical examiner familiar with rarely encountered heritable cardiac diseases causing SCA when pediatric SCA occurs. Procurement of and retention of DNA-bearing tissue for subsequent molecular autopsy should be encouraged for autopsy-negative cases.

    6. Support education programs for effective bystander CPR and appropriate AED use.

    7. Support development of effective school emergency response programs.

    8. Consider participation in school emergency response programs as a medical director.

    9. Support efforts to mandate a central registry for pediatric SCA as a reportable event.

    10. Support recommendation for evidence-based evaluation of national screening processes and programs.

    Lead Authors

    Robert Campbell, MD

    Stuart Berger, MD

    Michael J. Ackerman, MD, PhD

    Section on Cardiology and Cardiac Surgery Executive Committee, 2011–2012

    W. Robert Morrow, MD, Chairperson

    Stuart Berger, MD

    Kathy Jenkins, MD

    L. LuAnn Minich, MD

    Geoffrey L. Rosenthal, MD, PhD

    Christopher S. Snyder, MD

    James Twedell, MD

    Past Executive Committee Members

    Robert H. Beekman III, MD, Immediate Past Chairperson

    Thomas S. Klitzner, MD, PhD, Past Chairperson

    Peter B. Manning, MD

    Jeffrey A. Towbin, MD

    Seema Mital, MD

    Staff

    Lynn Colegrove, MBA

    Footnotes

    • This document is copyrighted and is property of the American Academy of Pediatrics and its Board of Directors. All authors have filed conflict of interest statements with the American Academy of Pediatrics. Any conflicts have been resolved through a process approved by the Board of Directors. The American Academy of Pediatrics has neither solicited nor accepted any commercial involvement in the development of the content of this publication.

    • All policy statements from the American Academy of Pediatrics automatically expire 5 years after publication unless reaffirmed, revised, or retired at or before that time.

    References

    1. ↵
      1. Kung HC,
      2. Hoyert DL,
      3. Xu J,
      4. Murphy SL
      . Deaths: final data for 2005. Natl Vital Stat Rep. 2008;56(10):1–120pmid:18512336
      OpenUrlPubMed
    2. ↵
      1. Corrado D,
      2. Basso C,
      3. Schiavon M,
      4. Thiene G
      . Screening for hypertrophic cardiomyopathy in young athletes. N Engl J Med. 1998;339(6):364–369pmid:9691102
      OpenUrlCrossRefPubMed
      1. Driscoll DJ,
      2. Edwards WD
      . Sudden unexpected death in children and adolescents. J Am Coll Cardiol. 1985;5(suppl 6):118B–121Bpmid:3998328
      OpenUrlCrossRefPubMed
      1. Wren C
      . Sudden death in children and adolescents. Heart. 2002;88(4):426–431pmid:12231612
      OpenUrlFREE Full Text
      1. Neuspiel DR,
      2. Kuller LH
      . Sudden and unexpected natural death in childhood and adolescence. JAMA. 1985;254(10):1321–1325pmid:4021009
      OpenUrlCrossRefPubMed
    6. ↵
      1. Shen WK,
      2. Edwards WD,
      3. Hammill SC,
      4. Bailey KR,
      5. Ballard DJ,
      6. Gersh BJ
      . Sudden unexpected nontraumatic death in 54 young adults: a 30-year population-based study. Am J Cardiol. 1995;76(3):148–152pmid:7611149
      OpenUrlCrossRefPubMed
    7. ↵
      1. Spurgeon D
      . Sudden cardiac deaths rise 10% in young Americans. BMJ. 2001;322(7286):573pmid:11265663
      OpenUrlFREE Full Text
    8. ↵
      1. SoRelle R
      . Jump in sudden deaths reported in younger people during past decade. Circulation. 2001;103(10):e9019–e9021
      OpenUrlFREE Full Text
    9. ↵
      1. Maron BJ,
      2. Epstein SE,
      3. Roberts WC
      . Causes of sudden death in competitive athletes. J Am Coll Cardiol. 1986;7(1):204–214pmid:3510233
      OpenUrlCrossRefPubMed
    10. ↵
      1. Maron BJ,
      2. Doerr JJ,
      3. Haas TS,
      4. Tierney DM,
      5. Mueller FO
      . Abstract 3872: Profile and frequency of sudden death in 1463 young competitive athletes: from a 25 year U.S. national registry: 1980-2005. Circulation. 2006;114:II830pmid:16908774
      OpenUrlCrossRefPubMed
    11. ↵
      1. Maron BJ,
      2. Doerer JJ,
      3. Haas TS,
      4. Tierney DM,
      5. Mueller FO
      . Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980-2006. Circulation. 2009;119(8):1085–1092pmid:19221222
      OpenUrlAbstract/FREE Full Text
    12. ↵
      1. Corrado D,
      2. Basso C,
      3. Pavei A,
      4. Michieli P,
      5. Schiavon M,
      6. Thiene G
      . Trends in sudden cardiovascular death in young competitive athletes after implementation of a preparticipation screening program. JAMA. 2006;296(13):1593–1601pmid:17018804
      OpenUrlCrossRefPubMed
    13. ↵
      1. Corrado D,
      2. Basso C,
      3. Rizzoli G,
      4. Schiavon M,
      5. Thiene G
      . Does sports activity enhance the risk of sudden death in adolescents and young adults? J Am Coll Cardiol. 2003;42(11):1959–1963pmid:14662259
      OpenUrlCrossRefPubMed
    14. ↵
      1. Berger S,
      2. Kugler JD,
      3. Thomas JA,
      4. Friedberg DZ
      . Sudden cardiac death in children and adolescents: introduction and overview. Pediatr Clin North Am. 2004;51(5):1201–1209pmid:15331279
      OpenUrlCrossRefPubMed
    15. ↵
      1. Campbell RM,
      2. Berger S,
      3. Drezner J
      . Sudden cardiac arrest in children and young athletes: the importance of a detailed personal and family history in the pre-participation evaluation. Br J Sports Med. 2009;43(5):336–341pmid:18718974
      OpenUrlAbstract/FREE Full Text
    16. ↵
      1. Keren A,
      2. Syrris P,
      3. McKenna WJ
      . Hypertrophic cardiomyopathy: the genetic determinants of clinical disease expression. Nat Clin Pract Cardiovasc Med. 2008;5(3):158–168pmid:18227814
      OpenUrlCrossRefPubMed
    17. ↵
      1. Lehnart SE,
      2. Ackerman MJ,
      3. Benson DW Jr,
      4. et al
      . Inherited arrhythmias: a National Heart, Lung, and Blood Institute and Office of Rare Diseases workshop consensus report about the diagnosis, phenotyping, molecular mechanisms, and therapeutic approaches for primary cardiomyopathies of gene mutations affecting ion channel function. Circulation. 2007;116(20):2325–2345pmid:17998470
      OpenUrlAbstract/FREE Full Text
    18. ↵
      1. Behr E,
      2. Wood DA,
      3. Wright M,
      4. et al.,
      5. Sudden Arrhythmic Death Syndrome Steering Group
      . Cardiological assessment of first-degree relatives in sudden arrhythmic death syndrome. Lancet. 2003;362(9394):1457–1459pmid:14602442
      OpenUrlCrossRefPubMed
    19. ↵
      1. Tester DJ,
      2. Ackerman MJ
      . Postmortem long QT syndrome genetic testing for sudden unexplained death in the young. J Am Coll Cardiol. 2007;49(2):240–246pmid:17222736
      OpenUrlCrossRefPubMed
    20. ↵
      1. Tan HL,
      2. Hofman N,
      3. van Langen IM,
      4. van der Wal AC,
      5. Wilde AAM
      . Sudden unexplained death: heritability and diagnostic yield of cardiological and genetic examination in surviving relatives. Circulation. 2005;112(2):207–213pmid:15998675
      OpenUrlAbstract/FREE Full Text
    21. ↵
      1. Morales A,
      2. Cowan J,
      3. Dagua J,
      4. Hershberger RE
      . Family history: an essential tool for cardiovascular genetic medicine. Congest Heart Fail. 2008;14(1):37–45pmid:18256568
      OpenUrlCrossRefPubMed
    22. ↵
      1. Liberthson RR
      . Sudden death from cardiac causes in children and young adults. N Engl J Med. 1996;334(16):1039–1044pmid:8598843
      OpenUrlCrossRefPubMed
    23. ↵
      1. Wisten A,
      2. Messner T
      . Symptoms preceding sudden cardiac death in the young are common but often misinterpreted. Scand Cardiovasc J. 2005;39(3):143–149pmid:16146977
      OpenUrlCrossRefPubMed
    24. ↵
      1. Puranik R,
      2. Chow CK,
      3. Duflou JA,
      4. Kilborn MJ,
      5. McGuire MA
      . Sudden death in the young. Heart Rhythm. 2005;2(12):1277–1282pmid:16360077
      OpenUrlCrossRefPubMed
    25. ↵
      1. Leenhardt A,
      2. Lucet V,
      3. Denjoy I,
      4. Grau F,
      5. Ngoc DD,
      6. Coumel P
      . Catecholaminergic polymorphic ventricular tachycardia in children. A 7-year follow-up of 21 patients. Circulation. 1995;91(5):1512–1519pmid:7867192
      OpenUrlAbstract/FREE Full Text
    26. ↵
      1. Camfield CS,
      2. Camfield PR,
      3. Veugelers PJ
      . Death in children with epilepsy: a population-based study. Lancet. 2002;359(9321):1891–1895pmid:12057550
      OpenUrlCrossRefPubMed
    27. ↵
      1. Lear-Kaul KC,
      2. Coughlin L,
      3. Dobersen MJ
      . Sudden unexpected death in epilepsy: a retrospective study. Am J Forensic Med Pathol. 2005;26(1):11–17pmid:15725771
      OpenUrlCrossRefPubMed
    28. ↵
      1. Mukherjee S,
      2. Tripathi M,
      3. Chandra PS,
      4. et al
      . Cardiovascular autonomic functions in well-controlled and intractable partial epilepsies. Epilepsy Res. 2009;85(2-3):261–269pmid:19409754
      OpenUrlCrossRefPubMed
    29. ↵
      1. Adabag AS,
      2. Kuskowski MA,
      3. Maron BJ
      . Determinants for clinical diagnosis of hypertrophic cardiomyopathy. Am J Cardiol. 2006;98(11):1507–1511pmid:17126660
      OpenUrlCrossRefPubMed
    30. ↵
      1. Nugent AW,
      2. Daubeney PE,
      3. Chondros P,
      4. et al.,
      5. National Australian Childhood Cardiomyopathy Study
      . Clinical features and outcomes of childhood hypertrophic cardiomyopathy: results from a national population-based study. Circulation. 2005;112(9):1332–1338pmid:16116056
      OpenUrlAbstract/FREE Full Text
    31. ↵
      1. Basso C,
      2. Maron BJ,
      3. Corrado D,
      4. Thiene G
      . Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol. 2000;35(6):1493–1501pmid:10807452
      OpenUrlCrossRefPubMed
    32. ↵
      1. Zalzstein E,
      2. Hamilton R,
      3. Zucker N,
      4. Diamant S,
      5. Webb G
      . Aortic dissection in children and young adults: diagnosis, patients at risk, and outcomes. Cardiol Young. 2003;13(4):341–344pmid:14694954
      OpenUrlPubMed
    33. ↵
      1. Choi G,
      2. Kopplin LJ,
      3. Tester DJ,
      4. Will ML,
      5. Haglund CM,
      6. Ackerman MJ
      . Spectrum and frequency of cardiac channel defects in swimming-triggered arrhythmia syndromes. Circulation. 2004;110(15):2119–2124pmid:15466642
      OpenUrlAbstract/FREE Full Text
    34. ↵
      1. Ackerman MJ,
      2. Tester DJ,
      3. Porter CJ,
      4. Edwards WD
      . Molecular diagnosis of the inherited long-QT syndrome in a woman who died after near-drowning. N Engl J Med. 1999;341(15):1121–1125pmid:10511610
      OpenUrlCrossRefPubMed
    35. ↵
      1. Arnestad M,
      2. Crotti L,
      3. Rognum TO,
      4. et al
      . Prevalence of long-QT syndrome gene variants in sudden infant death syndrome. Circulation. 2007;115(3):361–367pmid:17210839
      OpenUrlAbstract/FREE Full Text
      1. Ackerman MJ,
      2. Siu BL,
      3. Sturner WQ,
      4. et al
      . Postmortem molecular analysis of SCN5A defects in sudden infant death syndrome. JAMA. 2001;286(18):2264–2269pmid:11710892
      OpenUrlCrossRefPubMed
      1. Tester DJ,
      2. Dura M,
      3. Carturan E,
      4. et al
      . A mechanism for sudden infant death syndrome (SIDS): stress-induced leak via ryanodine receptors. Heart Rhythm. 2007;4(6):733–739pmid:17556193
      OpenUrlCrossRefPubMed
    38. ↵
      1. Van Norstrand DW,
      2. Valdivia CR,
      3. Tester DJ,
      4. et al
      . Molecular and functional characterization of novel glycerol-3-phosphate dehydrogenase 1 like gene (GPD1-L) mutations in sudden infant death syndrome. Circulation. 2007;116(20):2253–2259pmid:17967976
      OpenUrlAbstract/FREE Full Text
    39. ↵
      1. Jervell A,
      2. Lange-Nielsen F
      . Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval and sudden death. Am Heart J. 1957;54(1):59–68pmid:13435203
      OpenUrlCrossRefPubMed
    40. ↵
      1. Probst V,
      2. Denjoy I,
      3. Meregalli PG,
      4. et al
      . Clinical aspects and prognosis of Brugada syndrome in children. Circulation. 2007;115(15):2042–2048pmid:17404158
      OpenUrlAbstract/FREE Full Text
    41. ↵
      1. Maron BJ,
      2. Shirani J,
      3. Poliac LC,
      4. Mathenge R,
      5. Roberts WC,
      6. Mueller FO
      . Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles. JAMA. 1996;276(3):199–204pmid:8667563
      OpenUrlCrossRefPubMed
    42. ↵
      1. Wilson MG,
      2. Basavarajaiah S,
      3. Whyte GP,
      4. Cox S,
      5. Loosemore M,
      6. Sharma S
      . Efficacy of personal symptom and family history questionnaires when screening for inherited cardiac pathologies: the role of electrocardiography. Br J Sports Med. 2008;42(3):207–211pmid:17717062
      OpenUrlAbstract/FREE Full Text
    43. ↵
      1. Glover DW,
      2. Maron BJ
      . Profile of preparticipation cardiovascular screening for high school athletes. JAMA. 1998;279(22):1817–1819pmid:9628714
      OpenUrlCrossRefPubMed
    44. ↵
      1. Maron BJ,
      2. Thompson PD,
      3. Ackerman MJ,
      4. et al.,
      5. American Heart Association Council on Nutrition, Physical Activity, and Metabolism
      . Recommendations and considerations related to preparticipation screening for cardiovascular abnormalities in competitive athletes: 2007 update: a scientific statement from the American Heart Association Council on Nutrition, Physical Activity, and Metabolism: endorsed by the American College of Cardiology Foundation. Circulation. 2007;115(12):1643–1655pmid:17353433
      OpenUrlFREE Full Text
    45. ↵
      1. Wapps JR
      , ed. Preparticipation Physical Examination. 3rd ed. Minneapolis, MN: McGraw-Hill Healthcare Information; 2005
    46. ↵
      1. Tasaki H,
      2. Hamasaki Y,
      3. Ichimaru T
      . Mass screening for heart disease of school children in Saga city: 7-year follow up study. Jpn Circ J. 1987;51(12):1415–1420pmid:3443998
      OpenUrlCrossRefPubMed
    47. ↵
      1. Haneda N,
      2. Mori C,
      3. Nishio T,
      4. et al
      . Heart diseases discovered by mass screening in the schools of Shimane Prefecture over a period of 5 years. Jpn Circ J. 1986;50(12):1325–1329pmid:3820545
      OpenUrlCrossRefPubMed
    48. ↵
      1. Schwartz PJ,
      2. Stramba-Badiale M,
      3. Segantini A,
      4. et al
      . Prolongation of the QT interval and the sudden infant death syndrome. N Engl J Med. 1998;338(24):1709–1714pmid:9624190
      OpenUrlCrossRefPubMed
    49. ↵
      International Olympic Committee Medical Commission, International Olympic Committee. Sudden cardiovascular death in sport: Lausanne recommendations. Adopted December 10, 2004. Available at: http://multimedia.olympic.org/pdf/en_report_886.pdf. Accessed February 3, 2011
    50. ↵
      1. Corrado D,
      2. Pelliccia A,
      3. Bjørnstad HH,
      4. et al.,
      5. Study Group of Sport Cardiology of the Working Group of Cardiac Rehabilitation and Exercise Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society of Cardiology,
      6. Consensus Statement of the Study Group of Sport Cardiology of the Working Group of Cardiac Rehabilitation and Exercise Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society of Cardiology
      . Cardiovascular pre-participation screening of young competitive athletes for prevention of sudden death: proposal for a common European protocol. Eur Heart J. 2005;26(5):516–524pmid:15689345
      OpenUrlAbstract/FREE Full Text
    51. ↵
      1. Fuller CM
      . Cost effectiveness analysis of screening of high school athletes for risk of sudden cardiac death. Med Sci Sports Exerc. 2000;32(5):887–890pmid:10795776
      OpenUrlCrossRefPubMed
    52. ↵
      1. Fuller CM,
      2. McNulty CM,
      3. Spring DA,
      4. et al
      . Prospective screening of 5,615 high school athletes for risk of sudden cardiac death. Med Sci Sports Exerc. 1997;29(9):1131–1138pmid:9309622
      OpenUrlCrossRefPubMed
    53. ↵
      1. Vetter VL,
      2. Elia J,
      3. Erickson C,
      4. et al.,
      5. American Heart Association Council on Cardiovascular Disease in the Young Congenital Cardiac Defects Committee,
      6. American Heart Association Council on Cardiovascular Nursing
      . Cardiovascular monitoring of children and adolescents with heart disease receiving medications for attention deficit/hyperactivity disorder [corrected]: a scientific statement from the American Heart Association Council on Cardiovascular Disease in the Young Congenital Cardiac Defects Committee and the Council on Cardiovascular Nursing [published correction appears in Circulation. 2009;120(7):e55–e59]. Circulation. 2008;117(18):2407–2423pmid:18427125
      OpenUrlFREE Full Text
    54. ↵
      1. Corrado D,
      2. McKenna WJ
      . Appropriate interpretation of the athlete’s electrocardiogram saves lives as well as money. Eur Heart J. 2007;28(16):1920–1922pmid:17623679
      OpenUrlFREE Full Text
    55. ↵
      1. Myerburg RJ,
      2. Vetter VL
      . Electrocardiograms should be included in preparticipation screening of athletes. Circulation. 2007;116(22):2616–2626pmid:18040041
      OpenUrlFREE Full Text
    56. ↵
      1. Chaitman BR
      . An electrocardiogram should not be included in routine preparticipation screening of young athletes. Circulation. 2007;116(22):2610–2614, discussion 2615pmid:18040040
      OpenUrlFREE Full Text
    57. ↵
      1. Tester DJ,
      2. Ackerman MJ
      . The role of molecular autopsy in unexplained sudden cardiac death. Curr Opin Cardiol. 2006;21(3):166–172pmid:16601452
      OpenUrlPubMed
    58. ↵
      1. Cummins RO,
      2. Ornato JP,
      3. Thies WH,
      4. Pepe PE
      . Improving survival from sudden cardiac arrest: the “chain of survival” concept. A statement for health professionals from the Advanced Cardiac Life Support Subcommittee and the Emergency Cardiac Care Committee, American Heart Association. Circulation. 1991;83(5):1832–1847pmid:2022039
      OpenUrlFREE Full Text
    59. ↵
      1. López-Herce J,
      2. García C,
      3. Domínguez P,
      4. et al.,
      5. Spanish Study Group of Cardiopulmonary Arrest in Children
      . Outcome of out-of-hospital cardiorespiratory arrest in children. Pediatr Emerg Care. 2005;21(12):807–815pmid:16340755
      OpenUrlCrossRefPubMed
      1. Tunstall-Pedoe H,
      2. Bailey L,
      3. Chamberlain DA,
      4. Marsden AK,
      5. Ward ME,
      6. Zideman DA
      . Survey of 3765 cardiopulmonary resuscitations in British hospitals (the BRESUS Study): methods and overall results. BMJ. 1992;304(6838):1347–1351pmid:1611332
      OpenUrlAbstract/FREE Full Text
    61. ↵
      1. Young KD,
      2. Seidel JS
      . Pediatric cardiopulmonary resuscitation: a collective review. Ann Emerg Med. 1999;33(2):195–205pmid:9922416
      OpenUrlCrossRefPubMed
      1. Donoghue AJ,
      2. Nadkarni VM,
      3. Elliott M,
      4. Durbin D,
      5. American Heart Assocation National Registry of Cardiopulmonary Resuscitation Investigators
      . Effect of hospital characteristics on outcomes from pediatric cardiopulmonary resuscitation: a report from the national registry of cardiopulmonary resuscitation. Pediatrics. 2006;118(3):995–1001pmid:16950990
      OpenUrlAbstract/FREE Full Text
    63. ↵
      1. Kuisma M,
      2. Suominen P,
      3. Korpela R
      . Paediatric out-of-hospital cardiac arrests—epidemiology and outcome. Resuscitation. 1995;30(2):141–150pmid:8560103
      OpenUrlCrossRefPubMed
    64. ↵
      1. Holmberg M,
      2. Holmberg S,
      3. Herlitz J
      . Effect of bystander cardiopulmonary resuscitation in out-of-hospital cardiac arrest patients in Sweden. Resuscitation. 2000;47(1):59–70pmid:11004382
      OpenUrlCrossRefPubMed
    65. ↵
      1. Swor R,
      2. Khan I,
      3. Domeier R,
      4. Honeycutt L,
      5. Chu K,
      6. Compton S
      . CPR training and CPR performance: do CPR-trained bystanders perform CPR? Acad Emerg Med. 2006;13(6):596–601pmid:16614455
      OpenUrlCrossRefPubMed
    66. ↵
      1. SOS-KANTO Study Group
      . Cardiopulmonary resuscitation by bystanders with chest compression only (SOS-KANTO): an observational study. Lancet. 2007;369(9565):920–926pmid:17368153
      OpenUrlCrossRefPubMed
    67. ↵
      1. Valenzuela TD,
      2. Kern KB,
      3. Clark LL,
      4. et al
      . Interruptions of chest compressions during emergency medical systems resuscitation. Circulation. 2005;112(9):1259–1265pmid:16116053
      OpenUrlAbstract/FREE Full Text
    68. ↵
      1. Schindler MB,
      2. Bohn D,
      3. Cox PN,
      4. et al
      . Outcome of out-of-hospital cardiac or respiratory arrest in children. N Engl J Med. 1996;335(20):1473–1479pmid:8890097
      OpenUrlCrossRefPubMed
    69. ↵
      1. Sirbaugh PE,
      2. Pepe PE,
      3. Shook JE,
      4. et al
      . A prospective, population-based study of the demographics, epidemiology, management, and outcome of out-of-hospital pediatric cardiopulmonary arrest. Ann Emerg Med. 1999;33(2):174–184pmid:9922413
      OpenUrlCrossRefPubMed
    70. ↵
      1. Hallstrom AP,
      2. Ornato JP,
      3. Weisfeldt M,
      4. et al.,
      5. Public Access Defibrillation Trial Investigators
      . Public-access defibrillation and survival after out-of-hospital cardiac arrest. N Engl J Med. 2004;351(7):637–646pmid:15306665
      OpenUrlCrossRefPubMed
    71. ↵
      1. Caffrey SL,
      2. Willoughby PJ,
      3. Pepe PE,
      4. Becker LB
      . Public use of automated external defibrillators. N Engl J Med. 2002;347(16):1242–1247pmid:12393821
      OpenUrlCrossRefPubMed
    72. ↵
      1. Valenzuela TD,
      2. Roe DJ,
      3. Nichol G,
      4. Clark LL,
      5. Spaite DW,
      6. Hardman RG
      . Outcomes of rapid defibrillation by security officers after cardiac arrest in casinos. N Engl J Med. 2000;343(17):1206–1209pmid:11071670
      OpenUrlCrossRefPubMed
    73. ↵
      1. Samson RA,
      2. Berg RA,
      3. Bingham R,
      4. Pediatric Advanced Life Support Task Force, International Liaison Committee on Resuscitation for the American Heart Association,
      5. European Resuscitation Council
      . Use of automated external defibrillators for children: an update—an advisory statement from the Pediatric Advanced Life Support Task Force, International Liaison Committee on Resuscitation. Pediatrics. 2003;112(1 pt 1):163–168pmid:12837882
      OpenUrlFREE Full Text
    74. ↵
      1. American Heart Association
      . 2005 American Heart Association (AHA) guidelines for cardiopulmonary resuscitation (CPR) and emergency cardiovascular care (ECC) of pediatric and neonatal patients: pediatric advanced life support. Pediatrics. 2006;117(5). Available at: www.pediatrics.org/cgi/content/full/117/5/e1005pmid:16651281
      OpenUrlFREE Full Text
    75. ↵
      1. Markenson D,
      2. Pyles L,
      3. Neish S,
      4. American Academy of Pediatrics Committee on Pediatric Emergency Medicine,
      5. American Academy of Pediatrics Section on Cardiology and Cardiac Surgery
      . Ventricular fibrillation and the use of automated external defibrillators on children. Pediatrics. 2007;120(5). Available at: www.pediatrics.org/content/120/5/1159pmid:17967922
      OpenUrlAbstract/FREE Full Text
    76. ↵
      1. Sapien RE,
      2. Allen A
      . Emergency preparation in schools: a snapshot of a rural state. Pediatr Emerg Care. 2001;17(5):329–333pmid:11673708
      OpenUrlCrossRefPubMed
    77. ↵
      1. Lotfi K,
      2. White L,
      3. Rea T,
      4. et al
      . Cardiac arrest in schools. Circulation. 2007;116(12):1374–1379pmid:17724258
      OpenUrlAbstract/FREE Full Text
    78. ↵
      1. Berger S,
      2. Whitstone BN,
      3. Frisbee SJ,
      4. et al
      . Cost-effectiveness of Project ADAM: a project to prevent sudden cardiac death in high school students. Pediatr Cardiol. 2004;25(6):660–667pmid:14743309
      OpenUrlCrossRefPubMed
    79. ↵
      1. Rothmier JD,
      2. Drezner JA,
      3. Harmon KG
      . Automated external defibrillators in Washington State high schools. Br J Sports Med. 2007;41(5):301–305pmid:17289857
      OpenUrlAbstract/FREE Full Text
    80. ↵
      1. Council on School Health
      . Medical emergencies occurring at school. Pediatrics. 2008;122(4):887–894pmid:18829817
      OpenUrlAbstract/FREE Full Text
    81. ↵
      1. Bardy GH,
      2. Lee KL,
      3. Mark DB,
      4. et al.,
      5. HAT Investigators
      . Home use of automated external defibrillators for sudden cardiac arrest. N Engl J Med. 2008;358(17):1793–1804pmid:18381485
      OpenUrlCrossRefPubMed
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    Pediatrics
    Vol. 129, Issue 4
    1 Apr 2012
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    Pediatric Sudden Cardiac Arrest
    SECTION ON CARDIOLOGY AND CARDIAC SURGERY
    Pediatrics Apr 2012, 129 (4) e1094-e1102; DOI: 10.1542/peds.2012-0144
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