Pediatric SCA, defined as the abrupt and unexpected loss of

heart function

"[Sudden] cardiac arrest is the sudden cessation of cardiac

activity so that the victim becomes unresponsive, with no
normal breathing and no signs of circulation. If corrective
measures are not taken rapidly, this condition progresses to
sudden death. Cardiac arrest should be used to signify an
event as described above, that is reversed, usually by CPR
and/or defibrillation or cardioversion, or cardiac pacing. Sudden
cardiac death should not be used to describe events that are
not fatal."
- 2006 ACC/AHA/HRS

In many cases of SCA/SCD, the cause is not identified. In a

prospective study of 490 cases of SCD in children and
young adults, 40 percent were unexplained. (Bagnall RD,
Weintraub RG, Ingles J, et al. A Prospective Study of Sudden
Cardiac Death among Children and Young Adults. N Engl J
Med 2016; 374:2441.)

When an etiology is identified, common causes include:

a. Primary arrhythmia/channelopathy (22 percent), including:

o Long QT syndrome
o Wolff-Parkinson-White syndrome
o Brugada syndrome
o Short QT syndrome
o Catecholaminergic polymorphic ventricular
tachycardia
b. Myocarditis (7 to 35 percent)
c. Cardiomyopathy (16 to 20 percent)
o hypertrophic cardiomyopathy, dilated *17 other, including use of illicit drugs and stimulants (eg,
cardiomyopathy, restrictive cardiomyopathy, left cocaine, ephedra) or prescription medications (eg,
ventricular noncompaction cardiomyopathy, and erythromycin, ketoconazole, carbamazepine).
arrhythmogenic cardiomyopathy
d. Congenital heart disease (CHD, 15 percent) The Following 4 appear to represent more ominous positive
o such as tetralogy of Fallot, hypoplastic left heart responses (based on expert opinion):
syndrome, and transposition of the great arteries. 1. Have you ever fainted, passed out, or had a seizure
e. Premature coronary artery disease was reported in some suddenly and without warning, especially during
case series that included young adults (ie, 30 to 35 years exercise or in response to auditory triggers such as
old); however, this is an uncommon cause of SCA/SCD in doorbells, alarm clocks, and ringing telephones?
patients <25 years old. 2. Have you ever had exercise-induced chest pain or
f. Other cardiac disorders (4 to 8 percent), including shortness of breath?
coronary arteritis, anomalous origin of coronary arteries, 3. Are you related to anyone with sud- den, unexplained,
aortic dissection, pulmonary hypertension, and mitral valve and unexpected death before the age of 50?
prolapse; however, the relationship between mitral valve 4. Are you related to anyone who has been diagnosed
prolapse and SCD is uncertain. with a sudden death–predisposing heart condition
g. Unspecified cardiac disease (7 percent). such as HCM, LQTS, Brugada syndrome, and so
forth? (See Table 1.)

Causes of SCA/SCD vary somewhat by age

 In young children (<2 years old), CHD is a major
cause, while primary arrhythmia and myocarditis are
less common.
 In adolescents and young adults, primary arrhythmia,
cardiomyopathy, and myocarditis are more common
and CHD is a less common cause of SCA/SCD.
Premature atherosclerotic coronary artery disease is
an uncommon cause of SCA/SCD in patients <25
years old.
 Children and adolescents can have coronary disease
from Kawasaki disease or anomalous origin of the
coronary arteries, which may cause SCD.
References: Jefferies, LJ. Sudden cardiac arrest and death in children. In: UpToDate, Triedman, KJ (Ed), UpToDate, Sep 19, 2019.
Section on Cardiology and Cardiac Surgery. Pediatric sudden cardiac arrest. Pediatrics 2012;129:e1094–e1102