key points
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A local news report of the sudden death of an apparently healthy child can devastate a community and fill a pediatrician's
office with worried families. Approximately half of pediatric and young adult patients who succumb to sudden cardiac death
(SCD) experience prodromal symptoms, such as actual or near syncope or chest pain.1 For the other half of victims, SCD may be the first "sign" of a life-threatening disease. Health-care providers should screen
for SCD risk factors at any age and opportunity, not just when community awareness is heightened.
Because there is no national registry of pediatric SCD, the exact number of cases of unexplained SCD among children and adolescents
in the United States is unknown, but a reasonable estimate is about 500 episodes a year.2 Each case of SCD provokes an emotional response and soul searching. Could this have been predicted? Could it have been prevented?
In many cases, the answer to one or both of these questions is "Yes."
Table 1 Causes of sudden cardiac death
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Why are they dying?
Most cases of pediatric sudden death are cardiac in nature, and every one is the result of an uncommon diagnosis (see Table
1). Causes of SCD can be grossly divided into structural-functional, primary electrical, and "other." In one study,3 hypertrophic cardiomyopathy accounted for approximately 36% of SCD cases. Anomalous origin of the left coronary artery from
the right coronary cusp (with the coronary vessel coursing between the aorta and pulmonary artery) accounted for another 19%.3 Long QT syndrome affects one in 5,000 to 7,000 people and has increasingly been recognized in the pediatric age group.4 The "other" causes of SCD include commotio cordis and abuse of certain drugs and stimulants, such as ephedra and cocaine.
All of the conditions listed in Table 1 can lead to ventricular fibrillation. Many of the causes of SCD are genetic or familial,
making investigation of the first affected family member critical for unraveling further familial involvement.
Uncovering a patient at risk of SCD
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Primary prevention: Screening, treatment, awareness
The assessment form lists questions for the patient and family history that may reveal a risk of SCD. These questions are
part of a comprehensive preparticipation athletic evaluation, but they are important for any active child. They should also
be asked in the emergency room, especially when patients have been brought in with seizure, syncope, complex ventricular arrhythmia,
or palpitations. Such an occasion may be the only opportunity to identify a patient or family member at risk of pediatric
SCD and to refer for evaluation.
When obtaining a family history, make it thorough, document the source, and update that history periodically. Often, parents
and children will not provide relevant information unless specifically asked. Nearly all Americans believe family health knowledge
is important, but only one third of families have written a family medical history to share with their relatives.5 The US Surgeon General launched the family history initiative in November 2004; a computerized tool found at www.hhs.gov/familyhistory/download.html can aid in organizing a family history, especially during family gatherings.
Your physical examination of the patient may reveal abnormal blood pressure or pulses, a heart murmur, arrhythmia, or physical
stigmata of Marfan syndrome. In patients with Loeys-Dietz syndrome, a familial form of heart disease that causes changes in
the aorta similar to those of Marfan syndrome, the presence of a bifid uvula should raise suspicion of the diagnosis.6But many patients at risk of SCD do not have significant findings on physical exam. Close attention to the patient and family
history is, therefore, key.
If you suspect a problem based on patient and family history or physical exam findings, refer the patient to a pediatric cardiologist
with expertise in diagnosing and managing the uncommon cardiac disorders that cause SCD. Once a diagnosis is confirmed, antiarrhythmic
drug therapy, family education, and activity restriction are possible. Newer cause-specific technologies such as radiofrequency
ablation and the automatic implantable cardioverter defibrillator (AICD) are being used more often.7
In addition to maintaining a high level of suspicion for a cardiac condition, you have an opportunity to educate families
and your local community about warning signs and how to prevent SCD in children. Tools such as awareness education and comprehensive
preparticipation athletic evaluation (PAE) for student athletes are the foundations of primary prevention. Yet many states
do not mandate a PAE that includes pertinent history questions.8 Primary care providers, sports medicine practitioners, and emergency physicians can encourage state legislatures, high-school
athletic associations, school nurses, parent-teacher associations, and parent booster clubs to adopt a comprehensive form.
A sample of the preparticipation athletic evaluation form that is endorsed by the American Academy of Pediatrics (AAP), American
Academy of Family Physicians, American College of Sports Medicine, American Medical Society for Sports Medicine, American
Orthopaedic Society for Sports Medicine, and American Osteopathic Academy of Sports Medicine can be found at www.aap.org/sections/sportsmedicine/spmedeval.pdf. The 2005 edition of the monograph Preparticipation Physical Evaluation includes coverage of cardiovascular evaluation, obesity, diabetes, disabled athletes, and sport-specific testing.8 [Editor's note: For a detailed review of this topic, see "Making the preparticipation athletic evaluation more than just a sports physical" in the September 2003 issue of Contemporary Pediatrics.]
Routine electrocardiogram and echocardiogram screening for athletes is not cost-effective and can lead to misdiagnosis as well as false-positive and false-negative results.9 These tests should be used at the discretion of the student's health-care provider based on the findings of the history and
physical exam. Comprehensive evaluation of patients and families judged to be at high risk is a more appropriate diagnostic
strategy for all of the causes of SCD listed in Table 1.
Secondary prevention: Resuscitation in the field
Because not all victims of SCD have a positive history or warning signs, cardiopulmonary resuscitation (CPR) and defibrillation
within three to five minutes of arrest is the key to secondary prevention of SCD. CPR training and comprehensive programs
on the use of an automated external defibrillator (AED) are proliferating in schools as part of public access defibrillation
(PAD) efforts around the country. Such programs could also benefit adults who work at a school or are there for other reasons
(e.g., student visits, sports and arts events, continuing education) if they should suffer a cardiac emergency.
Project ADAM (Automated Defibrillators in Adam's Memory) with Children's Hospital of Wisconsin and Project S.A.V.E. (Sudden
Cardiac Death: Awareness, Vision for Prevention and Education) with Children's Healthcare of Atlanta are examples of initiatives
to help schools implement a prevention program. Project ADAM, which started in 1999 after the sudden cardiac death of Adam
Lemel, a Wisconsin high-school athlete, has been operating for several years in that state and is offering affiliation opportunities
to get programs started in other areas. Contact the Project ADAM administrator at 414-266-3889 for more information.
Project S.A.V.E. offers free consultation to Georgia schools on the implementation of new AED programs, as well as evaluation
of existing programs. The consultation includes prevention and awareness strategies and emergency response planning for sudden
cardiac events. Advocating for CPR training for students is another important goal of both projects. Contact the Project S.A.V.E.
coordinator at 404-785-7201 for more information.
Community physicians can advocate for programs like these and volunteer their expertise in medical direction for local school
and district programs to prevent SCD. Medical direction may include oversight of medical aspects of the program, review of
policy and procedure for rescuer response, help with post-event review, and assistance with practice drills and other quality
assurance activities.
Preparing schools for medical emergencies
In January 2004, the American Heart Association (AHA) introduced a public health initiative—the Medical Emergency Response
Plan for Schools—endorsed by the AAP, the American College of Emergency Physicians, the National Association of School Nurses,
and the American Red Cross.10 The initiative encourages schools to develop and practice plans to respond to life-threatening medical emergencies. Five
core elements of a school emergency medical response plan are identified:10
Effective and efficient communication throughout the school campus. Linking all parts of the campus, including field houses, modular classrooms, and practice and playing fields, enables school
personnel or students to activate the EMS system immediately when an emergency occurs.
A coordinated and practiced response plan. This plan should be developed in concert with the school nurse, school or athletic team physicians, athletic trainers, and
local EMS providers.
Risk reduction. This includes safety precautions to prevent injuries and identification of students and staff with medical conditions that
may result in sudden cardiac arrest.
Table 2 Why emergency training and equipment is needed in schools
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Training and equipment for first aid and CPR. The AHA statement recommends that several teachers be trained as CPR and first aid instructors and that all high-school students
receive training in basic first aid and CPR. The need for such training is clear (see Table 2).
Implementation of a lay rescuer AED program in schools with an established need. Criteria for "established need" are:
- A reasonable probability of AED use within five years or an incident necessitating such use within the past five years;
- A child or adult in the school is believed to be at risk of sudden cardiac arrest; or
- An EMS call-to-shock time (interval from EMS notification to defibrillator placement) of less than five minutes cannot be
achieved reliably with conventional EMS services, and a collapse-to-shock time (interval from victim collapse to defibrillator
placement) of less than five minutes can be achieved reliably by training and equipping lay first responders.
Five elements are critical to the success of a lay rescuer AED program:
- medical or health-care provider oversight
- training of anticipated rescuers in CPR and use of an AED, including updates as needed
- coordination with the local EMS system
- appropriate device maintenance
- ongoing quality improvement, with practice drills and incident assessment.10
Call to action—this means you!
Resources
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Even though the number of pediatric victims of SCD is small, these deaths devastate families and communities. Our goal should
be: No deaths from pediatric SCD.
In our opinion, every child should have a "risk assessment" history obtained at regular intervals during well-child visits
and school physicals and on the occasion of any emergency room or specialty visits for seizures, syncope, and exercise-induced
asthma. We all have an obligation to support or spearhead prevention initiatives and PAD programs in our communities. School
staff and coaches of community youth sports should know the warning signs of SCD and the importance of timely emergency response.
CPR training should be encouraged for school staff and students. Increased awareness of sudden cardiac death—incidence, causes,
recognition, and emergency response—and the involvement of schools and communities in prevention will save lives.
MS. ELLISON is a school nurse consultant and Project S.A.V.E. coordinator, Community Health Development and Advocacy, Children's
Healthcare of Atlanta.
DR. RIEHLE is a pediatric cardiology fellow, Sibley Heart Center, Children's Healthcare of Atlanta.
DR. BERGER is professor of pediatrics, Medical College of Wisconsin, Milwaukee, and medical director, Herma Heart Center,
Children's Hospital of Wisconsin, Milwaukee.
DR. CAMPBELL is director of Sibley Heart Center at Children's Healthcare of Atlanta, and director of cardiology, department
of pediatrics, Emory University School of Medicine, Atlanta.
The authors have nothing to disclose in regard to affiliations with, or financial interests in, any organization that may
have an interest in any part of this article.
REFERENCES
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3. Maron BJ, Shirani J, Poliac LC, et al: Sudden death in young competitive athletes: Clinical, demographic, and pathological
profiles. JAMA 1996;276:199
4. Vincent GM: The long QT and Brugada syndromes: Causes of unexpected syncope and sudden cardiac death in children and young
adults. Seminars in Pediatric Neurology 2005;12(1):15
5. Yoon PW, Scheuner MT, Gwinn M, et al: Awareness of family health history as a risk factor for disease – United States,
2004. MMWR 2004;53(44):1044
6. Facial features clue to new syndrome. www.hopkinschildren.org/pages/news/CCNSpring05/patient.html. Accessed Sept. 9, 2005
7. Campbell RM: The treatment of cardiac causes of sudden death, syncope and seizure. Seminars in Pediatric Neurology 2005;12(1):59
8. Wappes JR (ed): Preparticipation Physical Evaluation, ed 3. Minneapolis, Minn., McGraw-Hill 2005
9. Seto CK: Preparticipation cardiovascular screening. Clinics in Sports Medicine 2003;22(1):23
10. Hazinski MF, Markenson D, Neish S, et al: Response to cardiac arrest and selected life-threatening medical emergencies.
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