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Preventing Pediatric Sudden Cardiac Death: Where Do We Start?

Robert M. Campbell and Stuart Berger

Pediatrics 2006;118;802-804
DOI: 10.1542/peds.2006-0564

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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly

publication, it has been published continuously since 1948. PEDIATRICS is owned, published,
and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk
Grove Village, Illinois, 60007. Copyright © 2006 by the American Academy of Pediatrics. All
rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.

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Preventing Pediatric Sudden Cardiac Death: Where

Do We Start?
Robert M. Campbell, MDa, Stuart Berger, MDb

aChildren’s Healthcare of Atlanta Sibley Heart Center, Emory University School of Medicine, Atlanta, Georgia; bChildren’s Hospital of Wisconsin-Herma Heart Center,

Milwaukee, Wisconsin
The authors have indicated they have no financial relationships relevant to this article to disclose.

F IRST STEPS FIRST. Good medical practice begins with

good history. The details of a disciplined, compre-
hensive patient and family history may provide the first
analysis of DNA extracted from postmortem blood and
tissue samples identified novel mutations in ryanodine
receptor/calcium-release channels, which supported the
clues to a patient-specific medical diagnosis that directs a idea that catecholaminergic polymorphic ventricular
patient-specific medical treatment. Patient and family tachycardia was the cause of death.
history is critical for diagnosing diseases that cause pe- The first suggestion that a patient or family may be
diatric sudden cardiac death (SCD). affected with one of the disease processes predisposing to
Pediatric SCD is uncommon. It is estimated that 500 SCD is often obtained from a patient/family history. In a
to 1000 pediatric patients ⬍21 years old will suffer SCD busy clinic, at first glance, it may seem difficult to prior-
each year in the United States.1 However, each event is itize time for a comprehensive, accurate patient and
absolutely devastating to parents, families, neighbors,
family history. However, these details cannot be ignored.
communities, and health care providers. Could the
It is estimated that ⬃40% of pediatric patients who
death have been prevented? Could the diagnosis have
suffer an SCD event may have unrecognized or under-
been made before the fatal event?
appreciated warning symptoms. Specifically, patients
The differential diagnosis for causes of pediatric SCD
should be questioned about the history of syncope or
includes anatomic and structural abnormalities of the
heart (eg, hypertrophic cardiomyopathy, single right seizure during exercise, emotion, or startle; extreme fa-
coronary artery with anomalous course of the left coro- tigue associated with exercise (different from other chil-
nary between the aorta and pulmonary artery, arrhyth- dren); and/or unusual or extreme shortness of breath
mogenic right ventricular cardiomyopathy, etc), primary during exercise, often mimicking exercise-induced bron-
electrical cardiac disorders (including long QT syndrome, chospasm. Family issues include sudden, unexpected,
Brugada syndrome, catecholaminergic polymorphic unexplained death before the age of 50 (including sud-
ventricular tachycardia, short QT syndrome), stimulant den infant death syndrome, car accidents, or drowning);
use (eg, cocaine, ephedra), and trauma (commotio cor- family members with unexplained syncope or seizures;
dis). Importantly, because many of these disorders are congenital deafness; pacemakers or automatic implant-
genetic, the identification of a first-affected family mem-
ber may unravel extensive family involvement. Abbreviation: SCD, sudden cardiac death
The impact of genetic evaluation was detailed re-
Opinions expressed in this commentary are those of the authors and not necessarily those
cently in 2 publications. Tan et al2 reported on the diag- of the American Academy of Pediatrics or its Committees.
nostic yield of cardiac and genetic examination in sur- www.pediatrics.org/cgi/doi/10.1542/peds.2006-0564
viving relatives of probands with SCD. Examination of doi:10.1542/peds.2006-0564
relatives of SCD victims allowed identification of the Accepted for publication Mar 1, 2006
disease in 40% of the families and in 8.9 presymptomatic Address correspondence to Robert M. Campbell, MD, Sibley Heart Center Cardiology, 2835
Brandywine Rd, Suite 300, Atlanta, GA 30341. E-mail: campbellr@kidsheart.com
causes per family. In addition, Tester et al3 reported new PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2006 by the
insights obtained from a “molecular autopsy.” Genetic American Academy of Pediatrics


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able cardioverter defibrillators; and/or a known family preparticipation. Although many pediatric SCD events
history of any of the conditions that predispose to SCD. are associated with activity, they are not isolated to high
Phrasing questions about patient/family history is also school or college athletes. Infants and elementary
critical. Using questions such as “tell me about any mem- school– and middle school–aged patients still succumb to
ber of your family who has . . .” places the responsibility pediatric SCD and, likewise, should be screened. A form
on the parents and family to research the answers rather that includes pertinent cardiovascular risk-assessment
than simply providing immediate, and possibly incom- questions could be administered at periodic intervals
plete, responses. during the well-child continuum. Patients or families
Patients or families regarded as at higher risk for SCD deemed at higher risk because of positive responses on
on the basis of positive responses to these questions this questionnaire should be referred for thorough car-
should be referred to a pediatric cardiologist, who is diovascular evaluation.
trained in the diagnosis of diseases causing SCD, for a In summary, the first steps to preventing pediatric
thorough evaluation. The diagnosis of these rare disor- SCD include attention to the details of a comprehensive,
ders is often extremely difficult and may require exten- disciplined patient/family history. Warning signs should
sive testing, including genotyping, for certain disorders. be recognized and respected. The use of a standardized
Nearly all Americans believe that family health preparticipation evaluation form directs a comprehen-
knowledge is important, but only one third of families sive patient/family history. The development and wide-
have written a family medical history to share with their spread use of a cardiovascular risk-assessment form
relatives. In November 2004, the US Surgeon General would allow evaluation of any patient at any time. Any
launched a family-history initiative. A computerized patient with positive findings on patient and family his-
checklist (available at: www.hhs.gov/familyhistory/ tory should be considered for referral to a pediatric car-
download.html) can help families organize their history, diologist for comprehensive evaluation. Continuing
especially during family gatherings. medical education about the causes of pediatric SCD as
The American Academy of Pediatrics, American well as the warning signs and management strategies
Academy of Family Physicians, American College of should continue for all providers including pediatricians,
Sports Medicine, American Medical Society of Sports family practitioners, pediatric cardiologists, and emer-
Medicine, American Orthopedics Society of Sports Med- gency department physicians, as well as families and
icine, and American Osteopathic Academy of Sports school staff.
Medicine recently endorsed a standardized preparticipa- If we reemphasize the basics of good medicine (the
tion athletic evaluation form (available at: www.aap. patient and family history), we will be on the way with
org/sections/sportsmedicine/spmedeval.pdf). Questions the first steps to preventing pediatric SCD.
5 through 14 on this standardized form deal with issues
of cardiovascular risk assessment. To successfully screen
for cardiovascular risk, these questions must be admin-
1. Berger S, Kugler JD, Thomas JA, Friedberg DZ. Sudden cardiac
istered carefully. Families must understand the impor- death in children and adolescents: introduction and overview.
tance of obtaining accurate, detailed information as this Pediatr Clin North Am. 2004;51:1201–1209
portion of the form is completed. 2. Tan H, Hofman N, van Langen I, van der Wal A, Wilde A.
We propose the development of a standardized car- Sudden unexplained death: heritability and diagnostic yield of
diovascular risk-assessment form, which could be used cardiological and genetic examination in surviving relatives.
Circulation. 2005;112:207–213
by any provider, for any child, at any age, at any time 3. Tester D, Kopplin L, Creighton W, Burke A, Ackerman M.
(see Appendix). Identification of possible cardiovascular Pathogenesis of unexplained drowning: new insights from a
risk need not be obtained only during times of athletic molecular autopsy. Mayo Clin Proc. 2005;80:596 – 600

PEDIATRICS Volume 118, Number 2, August 2006 803

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To assess the risk of sudden cardiac death, ask these questions (or have parents complete this form) at periodic well-child visits: neonatal, preschool, before and
during middle school, and before and during high school.
Note for healthcare providers: When reviewing this form with parents/families, use phrases such as, “Tell me about any family member who
has . . .”
Yes No
Patient history questions
Has your child fainted or passed out DURING exercise, emotion, or startle?
Has your child fainted or passed out AFTER exercise?
Has your child had extreme fatigue associated with exercise (different from other children)?
Has your child ever had unusual or extreme shortness of breath during exercise?
Has your child ever had discomfort, pain, or pressure in his/her chest during exercise or complained of his/her heart “racing or skipping
Has a doctor ever told you that your child has high blood pressure, high cholesterol, a heart murmur, or a heart infection? (If “yes,” check
which one[s])
Has a doctor ever ordered a test for your child’s heart?
Has your child ever been diagnosed with an unexplained seizure disorder or exercise-induced asthma?
Family history questions
Are there any family members who had a sudden, unexpected death before age 50 (including from sudden infant death syndrome [SIDS],
car accident, drowning, or others)?
Are there any family members who died suddenly of “heart problems” before age 50?
Are there any family members who have had unexplained fainting or seizures?
Are there any relatives with certain conditions such as:
Hypertrophic cardiomyopathy (HCM)
Dilated cardiomyopathy (DCM)
Aortic rupture or Marfan syndrome
Coronary artery atherosclerotic disease (heart attack, age 50 years or younger)
Arrhythmogenic right ventricular cardiomyopathy (ARVC)
Long QT syndrome (LQTS)
Short QT syndrome
Brugada syndrome
Catecholaminergic polymorphic ventricular tachycardia (CPVT)
Primary pulmonary hypertension
Pacemaker or implanted cardiac defibrillator
Congenital deafness (deaf at birth)
Please explain any “yes” answers here:


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Preventing Pediatric Sudden Cardiac Death: Where Do We Start?
Robert M. Campbell and Stuart Berger
Pediatrics 2006;118;802-804
DOI: 10.1542/peds.2006-0564
Updated Information including high-resolution figures, can be found at:
& Services http://www.pediatrics.org/cgi/content/full/118/2/802
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