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Chest Pain in Pediatrics

Tisha K. Yeh, MD; and Jay Yeh, MD

Abstract
Tisha K. Yeh, MD, is an Attending Physi-
Chest pain in children and adolescents frequently involves referral to a pediatric cardi-
cian and Associate Physician Diplomate,
ologist. The etiology of chest pain in pediatrics is broad, and the vast majority of cases are
Division of Pediatric Hospital Medicine, Uni-
not due to underlying cardiac pathology. However, evaluations are often pursued due to versity of California Davis Children’s Hos-
fear about missing a potentially serious cardiac diagnosis, which may lead to sudden car- pital. Jay Yeh, MD, is an Assistant Professor
diac death. The management of these patients can lead to extensive investigations, medi- of Pediatrics, Division of Pediatric Cardiol-
cal visits, and hospitalizations, which is costly and unnecessary in many cases. This article ogy, University of California Davis Children’s
reviews noncardiac and cardiac etiologies of chest pain, highlights pertinent details of the Hospital.
patient history and physical examination, discusses the evaluation of patients with chest Address correspondence to Tisha K. Yeh,
pain, and identifies when referral to a pediatric cardiologist is recommended. [Pediatr Ann. MD, University of California Davis Children’s
2015;44(12):e274-e278.] Hospital, 2516 Stockton Boulevard, Sacra-
mento, CA 95817; email: tkyeh@ucdavis.
edu.
Disclosure: The authors have no relevant
financial relationships to disclose.
doi: 10.3928/00904481-20151110-01
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e274 Copyright © SLACK Incorporated


A
fter murmurs, chest pain is the
second most common reason
for referral to a pediatric car-
diologist.1 Pediatric patients with chest
pain account for at least 650,000 visits
annually in patients age 10 to 21 years.2,3
Patients with this primary complaint
account for 5.2% of all cardiology con-
sultations in inpatient and emergency
department settings4 and 15% of all out-
patient visits.5 Previous studies estimated
a cardiac etiology in only 0% to 5% of
patients with chest pain.5
Saleeb et al.6 reviewed the records of
3,700 patients seen in a pediatric cardiol-
ogy clinic for chest pain and found only
1% had a cardiac etiology. In this group,
the majority of patients were diagnosed
with noncardiac causes (99%), includ-
ing idiopathic (52%), musculoskeletal
(36%), respiratory (7%), and gastroin-
testinal (3%). Furthermore, this study
revealed that no patient discharged from
the cardiology clinic died as a result of
a cardiac condition over the 10-year pe-
riod examined and suggested that com-
plete histories, physical examinations,
and electrocardiograms are effective for
initial screening. Friedman et al.7 devel-
oped a standardized algorithm using this
approach, known as the SCAMP (Stan-
dardized Clinical Assessment and Man-
agement Plan) initiative for evaluation of
pediatric chest pain, and demonstrated a
reduction in resource utilization leading
to cost-effective care.8
NONCARDIAC CAUSES OF CHEST
PAIN
Noncardiac etiologies are, by far, the
most common cause of chest pain in pe-
diatrics. They can be classified as mus-
culoskeletal, pulmonary, gastrointestinal,
psychogenic, and idiopathic.
Musculoskeletal/Chest Wall
Pain related to the chest wall is typi-
cally the most common cause of noncar-
diac chest pain, with an estimated inci-
dence between 50% and 68%.10 Muscle
PEDIATRIC ANNALS • Vol. 44, No. 12, 2015
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strain and trauma can lead to such pain.
Strain of the chest wall muscles during
coughing can result in pain. Costochon-
dritis is due to inflammation at the cos-
tochondral junction, may be aggravated
by deep inspiration, may be preceded by
activity, and is typically reproducible on
Approximately 2% to
8% of pediatric chest
pain can be attributed to
gastrointestinal conditions.
palpation. Precordial catch syndrome is
typically brief, sharp pain localized at the
left side of the chest, and is sometimes
relieved by deep inspiration. Slipping
rib syndrome is caused by irritation of
the intercostal nerves when the inferior
false rib (ribs 8th, 9th, 10th) slips under
the adjacent rib. This may lead to pain
of the chest or upper abdomen. The diag-
nosis can be made using the “hooking”
maneuver, in which the examiner places
the fingers under the lower costal margin
and lifts anteriorly. This can reproduce
the pain and produce a clicking sensa-
tion. In sickle cell patients, acute chest
syndrome is characterized by chest pain,
hypoxemia, and a pulmonary infiltrate in
the setting of fever or respiratory symp-
toms. Skin conditions affecting the chest,
such as herpes zoster (shingles), can lead
to pain. Breast conditions are more com-
mon in girls and women and may cause
chest wall pain due to infection, puberty,
and pregnancy.
Pulmonary
The estimated incidence of noncardi-
ac chest pain related to respiratory causes
is 3% to 12%.5 Bronchospasm in reac-
tive airway disease and asthma can com-
monly cause chest pain in children and
is improved with bronchodilator therapy.
Pneumonia can cause chest pain, and the
presence of a fever and rales can sug-
gest this diagnosis. Pleuritic chest pain
can be characterized as sharp, worsened
by inspiration and coughing, and can be
referred to the shoulder from diaphrag-
matic irritation. Pneumothorax can cause
acute chest pain, may be secondary to
trauma, or may be spontaneous in condi-
tions such as Marfan syndrome and cys-
tic fibrosis.9 Pulmonary embolism can
also cause severe chest pain with associ-
ated shortness of breath and hypoxemia.
Gastrointestinal
Approximately 2% to 8% of pediat-
ric chest pain can be attributed to gas-
trointestinal conditions.5 Gastroesopha-
geal reflux and esophagitis can produce
retrosternal chest pain that may be ex-
acerbated by eating or by the supine
position. Peptic ulcer disease, gastritis,
esophageal foreign body, caustic inges-
tion, cholecystitis, and constipation are
other possible causes of noncardiac chest
pain.
Psychogenic
As much as 10% to 30% of chest pain
can be psychogenic in nature10 and is
more common in adolescents.5 A detailed
history may suggest a significant change
or social stressor as the precipitating fac-
tor. In patients with anxiety attacks, chest
pain may be present in hyperventilation
syndrome.9
Idiopathic
When an organic or psychogenic
cause cannot be determined, patients
may be considered to have idiopathic
chest pain.
CARDIAC CAUSES OF CHEST PAIN
Chest pain with exertion or associ-
ated palpitations, syncope, or decreased
endurance should raise suspicion of
cardiac pathology. Children with cer-
tain congenital heart defects, history
of heart transplant, substance abuse, or
e275

prior Kawasaki disease are at increased
risk for myocardial ischemia. A positive
family history of sudden unexplained
death, early heart attacks, cardiomyop-
athy, or cardiac arrhythmia should also
raise suspicion. Patients with concern-
ing histories and physical examination
results justify consultation with a pedi-
atric cardiologist for chest pain.
Aortic
Chest pain due to aortic pathology
is typically from aortic dissection. The
pain is classically described as sudden,
severe chest pain with a tearing or rip-
ping quality. Fortunately, dissection
of the aorta is rare in pediatrics. Most
cases of aortic dissection in children
and adolescents involve patients with
history of trauma or connective tissue
disorders such as Marfan syndrome,
Ehlers-Danlos syndrome, and Loeys-
Dietz syndrome. A retrospective analy-
sis10 over a 10-year period in New York
State found only 0.37% of cases of
aortic dissection were in patients age
21 years or younger; no patient was
younger than age 15 years. Trauma was
the most common associated condition
(42%) followed by Marfan syndrome
(24%). Other causes of aortic dissec-
tion are Turner syndrome, coarctation
of the aorta (repaired and unrepaired),
bicuspid aortic valve, familial thoracic
aortic aneurysm (TAAD), dissection,
and even metabolic disorders (eg, ho-
mocystinuria, familial hypercholester-
olemia).
Pericardial
Pericarditis is the acute inflamma-
tion of the pericardium and frequently
manifests with chest pain that is sharp,
increased with inspiration, improved
when seated and leaning forward,
worsened by lying flat, and occasion-
ally radiates to the left shoulder. Physi-
cal examination may demonstrate a
pericardial friction rub. Distant heart
sounds and/or pulsus paradoxus sug-
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gest a pericardial effusion associated
with the inflammation. Diagnostic test-
ing typically includes blood work, chest
X-ray, electrocardiogram (ECG), and
echocardiogram. Fever and new cardio-
megaly on chest X-ray should always
raise suspicion of infectious pericardi-
tis. An ECG classically demonstrates
A variety of acquired and
congenital coronary artery
disease is well recognized in
children.
new widespread ST elevation or PR
depression, followed later by T-wave
inversion. An echocardiogram may
identify location and size of associated
effusions. There are several underlying
causes of pericarditis: viral, bacterial,
or related to heart surgery. Pericarditis
can be a complication from collagen
vascular disorders, uremia, neoplasms,
or trauma. Inflammation of the adjacent
myocardium (myocarditis) should also
be considered when evaluating for peri-
carditis.
Coronary
A variety of acquired and congenital
coronary artery disease is well recog-
nized in children. Kawasaki disease is
a common pediatric vasculitis that may
result in the formation of coronary ar-
tery aneurysms. Coronary artery throm-
bosis and progressive stenosis within
the aneurysm may cause late ischemic
heart disease and chest pain. Treatment
with intravenous immunoglobulin dur-
ing the acute phase of Kawasaki dis-
ease reduces the prevalence of coro-
nary artery abnormalities.11 Although
uncommon, congenital coronary artery
abnormalities are well described in the
literature and may lead to chest pain.
Anomalous origin of a coronary artery
from the opposite sinus of Valsalva
(particularly when it courses between
the great arteries) has been associated
with myocardial ischemia, ventricular
arrhythmias, and sudden death. The
mechanisms that lead to myocardial
ischemia are unclear, but several theo-
ries have been proposed, from slit-like
orifices with compromised flow re-
serve, altered flow patterns due to the
acute angle of the coronary origin, and
deformation of the anomalous coro-
nary within the aortic wall (intramural
segment) during exercise.12 Anoma-
lous origin of the left coronary artery
from the pulmonary artery (ALCAPA)
usually presents in infancy, but can
be discovered later in childhood de-
pending on the amount of collateral
coronary blood flow. In ALCAPA, left
ventricular ischemia usually results in
cardiac dysfunction and mitral regur-
gitation.
Myocardial
Cardiomyopathies (most common-
ly hypertrophic or dilated) can be as-
sociated with chest pain. The pain is
due to excessive myocardial demand
and limited cardiac output, resulting
in angina. In hypertrophic cardio-
myopathy (HCM), mid-cavitary left
ventricular outflow tract obstruction
exacerbates the imbalance, leading to
increased myocardial workload and
oxygen consumption. In some HCM
patients, myocardial bridging may
produce coronary artery compres-
sion resulting in angina or sudden
death.13 In dilated cardiomyopathy,
although the myocardium does not
appear thickened, overall mass may
be abnormally high due to the signifi-
cant cardiomegaly. The impairment of
ventricular stroke volume exacerbates
the imbalance between myocardial de-
mands and coronary blood flow. Acute
myocarditis (most commonly viral
in etiology) may occasionally cause
Copyright © SLACK Incorporated

chest pain when accompanied by peri-
carditis.
Valvular
Severe aortic valve or subaortic ob-
struction produces chest pain in a simi-
lar fashion to HCM, where myocardial
metabolic demand exceeds a limited
cardiac output, leading to ischemia.
Mitral regurgitation, when severe, can
induce chest pain. Less dangerous and
poorly understood is chest pain in some
children with mitral valve prolapse,
which some suggest is unrelated to this
valve disorder.14
Rhythm Abnormalities
Children with any form of arrhyth-
mia (benign and serious) may com-
plain of chest pain during acute events.
In benign causes, the sensation of a
skipped beat or a more forceful sinus
beat after a premature atrial beat may
be regarded as painful. With more dan-
gerous arrhythmias, chest pain may be
the result of coronary ischemia due to
poor ventricular diastolic filling and
decreased cardiac output. Chest pain
from ventricular tachycardia is most
commonly seen in patients with his-
tory of open heart surgery, people with
cardiomyopathy, long QT syndrome,
other channelopathies, or severe elec-
trolyte disturbances.
EVALUATION
The patient history is critical to
determining the cause of chest pain.
Details should include a description
of the pain, including the quality, lo-
cation, severity, radiation, duration
from symptom onset, and precipitating
or aggravating factors. Chronic pain
is more associated with noncardiac
conditions. Other details such as im-
provement with rest or analgesics can
suggest alternative causes (musculo-
skeletal).
Concerning pertinent history in-
cludes exertional chest pain or ex-
PEDIATRIC ANNALS • Vol. 44, No. 12, 2015
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ertional syncope, palpitations as a
primary complaint along with chest
pain, radiation to the back, jaw, left
arm, or left shoulder, increased pain
while supine, or association with fe-
ver. Past medical history can be wor-
risome if the patient has a systemic
arthritis/vasculitis, a hypercoaguable
state, or a history of Kawasaki dis-
ease or cardiac surgery, which would
increase the risk of pathologic chest
pain. Family history is positive if a
first-degree relative has any of the fol-
lowing present: sudden or unexplained
death prior to age 50 years, cardiomy-
opathy, a hypercoaguable state, severe
familial hyperlipidemia, known famil-
ial arrhythmias, or pulmonary hyper-
tension.
Physical examination may reveal a
pathologic murmur, gallop, pericardial
friction rub, abnormal second heart
sound (loud or single S2), irregular
rhythm, distant heart sounds, peripher-
al edema, painful or swollen extremi-
ties, hypoxemia, ill appearance, tachy-
cardia, tachypnea, or fever.
Electrocardiographic findings that
are abnormal and suggest a cardiac
cause for chest pain are ventricular hy-
pertrophy, pathologic ST segment or T-
wave changes, high-grade atrioventric-
ular block, ventricular or atrial ectopy,
low QRS voltages, PR-segment depres-
sion, or a prolonged QTc >470 ms.8
Echocardiography may demonstrate
incidental findings but can diagnose
serious causes of chest pain, including
cardiomyopathies, left ventricular out-
flow obstruction, anomalous coronary
artery origin, pericardial effusion due
to pericarditis, and pulmonary hyper-
tension.5,9 Exercise stress testing and
ambulatory ECG are often low yield in
the evaluation of most cases of chest
pain in pediatrics.
SCAMP, a health care quality im-
provement initiative, has been devel-
oped to reduce practice variation, opti-
mize resource utilization, and improve
patient care.5,7,8 The SCAMP for pedi-
atric chest pain was created based on
chart review of 406 patients present-
ing to the pediatric cardiology clinic at
Boston Children’s Hospital for evalu-
ation of chest pain.7 The authors7 re-
viewed tests that included electrocar-
diography, echocardiography, exercise
stress testing, event monitoring, and
Holter monitoring. Significant medi-
cal or family history, abnormal ex-
amination, and/or abnormal ECG were
present in 11% of patients. Cardiac
etiology for chest pain was present
in 1.2% of patients (two patients with
pericarditis, three with arrhythmias).
By using history, physical examina-
tion, and ECG as screening measures,
the SCAMP algorithm was created to
identify when additional testing (ie,
echocardiogram) is indicated. This ap-
proach did not miss any cardiac diag-
noses responsible for chest pain in this
cohort.
REFERRAL
Referral to a pediatric cardiolo-
gist is recommended in patients with
concerning past medical history, fam-
ily history, abnormal examination,
or abnormal ECG. In the presence
of negative patient history, physical
examination, and ECG, an echocar-
diogram is indicated only in cases of
exertional chest pain with no alterna-
tive explanation.7 Referral to the emer-
gency department is recommended in
patients with fever and acute onset of
symptoms. Myocarditis and pericardi-
tis should be considered when fever,
abnormal vital signs, ill-appearance,
and a rub or gallop are present on ex-
amination.
CONCLUSION
Chest pain in children and adoles-
cents is most commonly due to non-
cardiac etiologies. Appropriate treat-
ment and referral of patients with
chest pain depends on the suspected
e277

etiology. Primary care physicians can
be reassured that most cardiac pathol-
ogy is excluded when the patient his-
tory, physical examination, and ECG
are normal.
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