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Advances in Pediatrics 70 (2023) 171–185

ADVANCES IN PEDIATRICS

Chest Pain, Palpitations, and


Syncope
Preventing Sudden Cardiac Death in Children

Saar Danon, MDa,b,*


a
Pediatric Cardiology and Congenital Cardiac Catheterization, Miller Children’s and Women’s
Hospital Long Beach, 2701 Atlantic Avenue, Long Beach, CA 90806, USA; bUniversity of Cali-
fornia, Irvine, CA, USA

Keywords
 Chest pain  Palpitations  Syncope  Cardiac arrest  Sudden cardiac death
Key points
 Most pediatric chest pain is noncardiac, but if it is exercise-related or associated
with syncope, cardiac etiology should be ruled out.
 Palpitations that start and stop suddenly and are associated with heart rate
greater than 150 are more likely to represent an arrhythmia.
 Syncope that is exercise-related, preceded by palpitations, or associated with
chest pain should increase suspicion for cardiac etiology.
 Exertional symptoms are more likely to represent cardiac pathology and warrant
further evaluation.

INTRODUCTION
Sudden cardiac death is defined as an abrupt, unexpected death of cardiovas-
cular cause with loss of consciousness within 1 hour of onset of symptoms
[1,2]. Aborted sudden death occurs if spontaneous circulation is restored,
which may lead to long-term morbidity [2]. In either situation, there is a
tremendous impact on the patient, their family, and the community. In an
effort to prevent these events, clinicians need to recognize symptoms to identify
at risk patients. This responsibility falls predominantly on primary care and
emergency physicians. There is often an overlap in symptoms of chest pain,

*Corresponding author. Pediatric Cardiology, 2701 Atlantic Avenue, Long Beach, CA


90806. E-mail address: SDanon@MemorialCare.org

https://doi.org/10.1016/j.yapd.2023.04.003
0065-3101/23/ª 2023 Elsevier Inc. All rights reserved.

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172 DANON

palpitations, and syncope [3,4]. The workup depends on the characteristics of


these symptoms.

GENERAL CONTENT
Patients may present with symptoms or as part of a routine check-up or sports
clearance. Box 1 is a screening tool which expands on previously published ta-
bles intended for screening of athletes [5,6]. This includes personal history,
family history, and physical examination. Any positive findings may warrant
further evaluation and possible referral to cardiology, particularly before
participating in sports.
The history includes questions related to cardiac symptoms, such as chest
pain, palpitations, dyspnea, and syncope/near-syncope. Symptoms that occur
primarily with exertion should raise concern [4,7]. In those situations, patients
should be restricted from playing sports and participating in other strenuous
activity until further evaluation. A prior history of Kawasaki disease, recent se-
vere acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, or

Box 1: Screening for pediatric cardiovascular disease[5,6]


Medical History
Personal History
1. Chest pain/discomfort related to exertion
2. Palpitations or excessive dyspnea related to exertion
3. Unexplained syncope/near-syncopea
4. History of moderate or severe SARS-CoV-2 infection (past 6 months)
5. History of Kawasaki disease or MIS-C
6. Previous heart murmur or cardiac testing
7. Elevated blood pressure/hypertension
8. Prior restriction from exercise by a physician
Family History
9. Congenital heart disease
10. Premature death or disability <50 y of age attributable to heart disease
11. Hypertrophic or dilated cardiomyopathy, long QT syndrome, other ion
channelopathies, Marfan syndrome or other connective tissue disorder,
arrhythmia, and other genetic cardiac conditions
Physical Examination
12. Heart murmurb
13. Absent or diminished femoral pulses
14. Physical stigmata of Marfan syndrome or other connective tissue disorder
15. Elevated blood pressure/hypertension
a
Judged not to be neurally mediated/vasovagal in origin; increased concern when occurring dur-
ing or after physical exertion.bNot consistent with innocent/physiologic murmur.

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CHEST PAIN, PALPITATIONS, AND SYNCOPE 173

multisystem inflammatory syndrome in children (MIS-C) should raise partic-


ular concern in a symptomatic patient due to risk of coronary aneurysm,
myocarditis, and other abnormalities [7–9]. Concerning family history includes
congenital heart disease, premature cardiac death or disability before age 50
years, arrhythmia, or known genetic cardiac disease such as cardiomyopathy,
long QT syndrome and other channelopathies, and Marfan syndrome and
other connective tissue disorders [5].

SYMPTOMS
Table 1 summarizes the symptom characteristics that warrant further cardiac
evaluation.

Chest pain
Patients often equate chest pain with a heart abnormality, despite cardiac chest
pain being rare in pediatrics. The history and physical are instrumental in
deciding whether further evaluation is warranted. Exertional, pressure-like
chest pain is most concerning and warrants an electrocardiogram (EKG) and
echocardiogram. If the pain is sharp, mostly at rest, and self-resolves, it is
much less likely to be cardiac. Other etiologies of chest pain include musculo-
skeletal, respiratory, gastrointestinal, and psychological [7,10].
For musculoskeletal chest pain, precordial catch should be differentiated
from costochondritis and other forms of chest wall inflammation. Precordial
catch is sharp, stabbing, sudden onset, occurs at rest, self-resolves within a

Table 1
Symptom characteristics requiring further cardiac evaluation
Symptom Require further cardiac evaluation Less-concerning characteristics
Chest pain Exercise-related Mostly at rest
Pressure-like, dull Sharp, stabbing, or burning
Associated with palpitations Short duration (seconds)
and/or syncope Related to meals
Self-resolving
Emotionally induced
Palpitations Exercise-related Gradual onset and termination
Start and stop suddenly Occur with orthostatic changes
Associated with near-syncope Preceded by feeling
or syncope lightheaded/dizzy
Heart rate >150 bpm Heart rate <150 bpm
Emotionally induced
Syncope Exercise-related Preceded by feeling
Preceded by palpitations lightheaded/dizzy
Associated with chest pain Following orthostatic changes
Auditory triggers Full and quick recovery
Seizure-like activity Situationala
a
Situational syncope includes syncope associated with coughing, swallowing, micturition, hair grooming,
blood draws, and other situations associated with vasovagal syncope.

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174 DANON

few minutes, and is sometimes worse with inspiration. There is no reproducible


chest pain on examination. Patients should be reassured, and no treatment is
needed. In costochondritis, there is inflammation of the chest wall leading to
reproducible chest pain with palpation [7,10]. These patients usually respond
to non-steroidal anti-inflammatory drugs (NSAIDs), typically a 5-day course.
It is important that it is given for this duration to decrease the inflammation
and not just as a response to pain.
Gastrointestinal chest pain is usually related to reflux and/or esophagitis.
The pain is substernal or upper abdominal, may involve a burning sensation,
and can be related to meals [7,10]. The pain often improves with dietary
changes to avoid spicy and fried foods and sometimes requires a trial of antacid
or referral to a gastroenterologist.
Respiratory chest pain may be related to asthma or exercise-induced bron-
choconstriction and may also be infectious in etiology [7,10]. If it is exercise
induced, asthma/bronchoconstriction is difficult to differentiate from cardiac
etiology. This may be achieved with an exercise treadmill study with pulmo-
nary function testing (PFT).
Psychological chest pain can typically be diagnosed based on the history and
normal cardiac examination. There may be a recent stressor, and the symp-
toms exacerbated by fear of a heart attack or other severe cardiac disease. Reas-
surance is helpful in alleviating anxiety related to the chest pain, and there
should be avoidance of unnecessary cardiac testing [7,10].

Palpitations
Palpitations are a common complaint in pediatrics. Some patients describe of a
skipped beat or funny feeling in their chest while at rest, lasting for a few sec-
onds which self-resolves. These symptoms are usually not associated with pa-
thology. If palpitations are longer in duration, an arrhythmia is more likely if
they start and terminate suddenly [4]. Young children may have difficulty
describing what they feel and complain of pain. Palpitations that precede
pre-syncope or syncope, as well as those brought on by exercise, are more
likely to be secondary to an arrhythmia. If the symptoms are related to an
emotional state, this is typically consistent with sinus tachycardia. Often it is
difficult to differentiate whether the anxiety preceded the symptoms or if it
was brought on as a result of feeling the palpitations.
It is helpful to know the heart rate during the symptoms. This used to be
dependent on capturing an episode with a cardiac monitor such as a Holter or
event recorder. With so many wearable devices, many patients have the ability
to check their heart rate during their symptoms. There are also free phone apps
which use the camera to monitor finger pulsations and measure a heart rate. If the
heart rate is less than 150 during the symptoms, an arrhythmia is much less likely
[4]. Parents and patients should download a heart rate app to check their heart
rate when experiencing symptoms. They should maintain a diary documenting
symptoms, approximate duration, associated activity, and heart rate. If there is
concern for an arrhythmia, a home cardiac monitoring study is indicated.

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CHEST PAIN, PALPITATIONS, AND SYNCOPE 175

Syncope
There is an overlap in the etiology and terminology of syncope, including
neurally mediated, vasovagal, neurocardiogenic, postural orthostatic tachycardia
syndrome, situational syncope (eg, related to coughing, swallowing, micturition,
hair grooming, blood draws), and other potential causes. If syncope occurs dur-
ing or immediately following exercise, then cardiac etiology needs to be ruled out
and the patient should not participate in exercise until further workup. If the syn-
cope is related to positional changes and there is a prodrome such as dizziness,
pallor, or diaphoresis, then neurally mediated syncope is more likely. If the syn-
cope occurs following palpitations, then an arrhythmia should be considered.
This is often difficult to ascertain as positional changes may lead to palpitations
related to orthostatic sinus tachycardia. Other concerning syncope characteristics
are associated chest pain, auditory triggers, or seizure-like activity. Positive family
history of cardiac disease such as long QT syndrome, other channelopathies,
arrhythmia, and cardiomyopathy should raise concern. EKG is indicated as
part of the workup, and unless there is a clear benign cause for the syncope,
then an echocardiogram is warranted [3].
Most often the neurally mediated causes of syncope may improve with
increased hydration (64–100 ounces of water per day), increased salt intake,
adequate diet without skipping meals, sleep of at least 9 hours per night, and
daily exercises with a goal of 1 hour per day. In addition, if patients feel pre-
syncopal, they should squat down, which may prevent syncope by increasing
preload and cardiac output. If they do faint, they are much less likely to be
injured from a squatting position. If these measures do not improve the symp-
toms, there are medications that may be used to help alleviate the symptoms.

Dyspnea
The shortness of breath or dyspnea is a common symptom although difficult to
differentiate from normal fatigue. This may be concerning if related to activity
and out of proportion to normal dyspnea related to exercise [5,6]. This is sub-
jective and difficult to ascertain. When obtaining a history, it is important to
ask whether this is a new or worsening symptom. These symptoms may
also be related to deconditioning. This is one of the sequalae seen as a result
of the COVID-19 pandemic and shut down of schools, sports, and other
activities.

EVALUATION AND WORKUP


Physical examination
The physical examination, including vital signs, may yield a lot of useful infor-
mation. Box 1 includes physical examination findings, which warrant further
evaluation. Blood pressure should be checked in the right arm using an appro-
priately sized cuff. If the blood pressure is elevated, it should be rechecked after
allowing the patient to be seated and rest for 5 minutes. A blood pressure
95th percentile is classified as hypertension [11]. In patients with orthostatic
symptoms, near-syncope, or syncope, orthostatic vitals should be assessed.

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176 DANON

The heart rate and blood pressure are measured following 5 minutes in the su-
pine position, and again 1 and 3 minutes after standing. Orthostatic tachycardia
is defined by an increase in heart rate of greater than 40 with standing, and
orthostatic hypotension is defined as a drop in systolic BP of 20 mm Hg
or diastolic BP of 10 mm Hg within 3 minutes of standing [3,12].
The cardiac examination includes inspection for scars and chest wall defor-
mities. Palpation may identify an increased right ventricular impulse, and
reproducible chest pain or tenderness. Auscultation is performed for rhythm
irregularity, murmurs, and other abnormal heart sounds. An innocent murmur
should be distinguished from a pathologic murmur when deciding whether to
refer to cardiology. A vibratory murmur which is louder in the supine position
is more likely to be physiologic. Auscultation should be performed in the su-
pine and standing positions or with a Valsalva maneuver. This may intensify
a murmur associated with dynamic left ventricular outflow tract (LVOT)
obstruction seen in hypertrophic cardiomyopathy. Both standing and perform-
ing a Valsalva maneuver acutely decrease preload, exacerbating the degree of
LVOT obstruction, which causes a more pronounced murmur. Pulses should
be felt in the right upper extremity and at least one lower extremity. Absent or
diminished femoral pulse or brachial–femoral delay warrants an echocardio-
gram to evaluate for coarctation of the aorta.
Physical signs of Marfan syndrome and other connective tissue disorders,
such as Louis–Dietz and Ehlers–Danlos, warrant further evaluation due to
their association with valvar abnormalities and aortic root dilation, which
carries a risk of aortic dissection [3,13,14]. Although significant valve abnormal-
ities will cause a murmur, patients with even severe aortic root dilation may
have a completely normal cardiac examination.
Electrocardiogram
EKG is usually the first test performed in evaluating patients with cardiac
symptoms. Although a normal EKG (Fig. 1) cannot rule out pathology, an
abnormal result can help guide further workup. It may identify the most com-
mon cause of sudden cardiac death in children, hypertrophic cardiomyopathy,
as well as long QT syndrome (Fig. 2), other ion channelopathies, and Wolff–

Fig. 1. EKG performed in a 12-year old boy with irregular rhythm noted on physical exam-
ination. The EKG demonstrates sinus arrhythmia. This is a normal finding of physiologic heart
rate variation with the respiratory cycle.

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CHEST PAIN, PALPITATIONS, AND SYNCOPE 177

Fig. 2. EKG of an 8-year old boy with syncope demonstrating findings of long QT syndrome.
There is significant prolongation of the QT interval. There is also T-wave alternans (asterisk), a
beat-to-beat variability in T-wave morphology, which has been associated with an increased
risk of ventricular tachyarrhythmias and sudden cardiac death. (Image borrowed with permis-
sion from Anjan Batra, MD, MBA, Professor of Pediatrics, University of California, Irvine.)

Parkinson–White syndrome. The EKG is also useful in post-viral myocarditis.


It is important that the EKG is read by a cardiologist experienced in reading pe-
diatric EKGs [15]. The automated read by the machine is often incorrect, and
adult cardiologists may not recognize findings typically seen in children [16].

Echocardiogram
An echocardiogram is a cardiac ultrasound often performed as part of the eval-
uation by a pediatric cardiologist. This is a noninvasive study that provides
both anatomic definition and functional assessment of the heart and great ves-
sels. A complete echocardiogram includes the assessment of systolic and dia-
stolic myocardial function, valvar abnormalities, shunt lesions, chamber
enlargement or thickening, aortic and pulmonary artery abnormalities, and cor-
onary artery anomalies. It is also used in the following progression of disease
once identified. Imaging quality and portability continues to improve, making
it the primary imaging modality in the diagnosis and management of cardiac
disease. Fig. 3 demonstrates echocardiographic findings in a patient who pre-
sented with an aborted sudden death episode and subsequently diagnosed
with hypertrophic cardiomyopathy.

Advanced cardiac imaging


Computed tomography (CT) and MRI are used to obtain cross-sectional im-
ages of the heart, which can be reconstructed into several different planes
and 3-dimensions. This is instrumental in making a correct anatomic diagnosis

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178 DANON

Fig. 3. Echocardiogram of a 13-year old boy who collapsed while running track. He was
noted to be in ventricular fibrillation when paramedics arrived and was defibrillated. Echocar-
diogram in two views, long axis (A) and short axis (B), demonstrating a concentrically thick-
ened myocardium consistent with hypertrophic cardiomyopathy. There is a small left
ventricular (LV) cavity and increased dimensions of the interventricular septum (IVS) and poste-
rior wall (PW). Ao, aorta; LA, left atrium.

Fig. 4. CT of anomalous aortic origin of the right coronary artery from the left coronary sinus
in a 14-year old boy who presented with chest pain while running. (A) 3D reconstruction
demonstrating the right coronary artery (RCA) originating from left coronary sinus and
coursing anterior to the aorta (Ao) toward the right. (B) In the cross-sectional image, the
RCA is seen coursing anterior and rightward between the Ao and the pulmonary artery
(PA), where it is at risk for compression during exercise. A, anterior; I, inferior; L, left; P, pos-
terior; R, right; S, superior. *NRCAO (normal right coronary artery origin) is the location
where the RCA normally originates. (Image borrowed with permission from Wilson King,
MD, Associate Professor of Pediatrics, Saint Louis University.)

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CHEST PAIN, PALPITATIONS, AND SYNCOPE 179

and in planning surgical or transcatheter interventions. These studies can also


be used to obtain moving images to demonstrate cardiac function and flow.
Cardiac CT uses ionizing radiation to obtain high-resolution images of cardiac
and vascular structures, including the coronary arteries (Fig. 4). Cardiac MRI
uses magnetic fields and radio waves to obtain the images; therefore, there is no
exposure to ionizing radiation. The MRI can provide anatomical and func-
tional data, as well as characterizing myocardial tissue, such as assessing for
myocardial inflammation seen in post-viral myocarditis (Fig. 5).

Home cardiac monitor


Wearable devices and phones are beneficial in identifying the heart rate during
symptoms, and some can even obtain a single-lead EKG. If further cardiac
monitoring is warranted, there are multiple home monitoring devices. These
have become much smaller and are now a single patch with the entire device
embedded and do not require multiple leads and wires. Patients wear these de-
vices from 24 hours up to 30 days in attempt to capture the episodes and make
a definitive diagnosis. Some have the option to include real-time telemetry, so a
physician is notified within minutes of a critical abnormality. The monitors pro-
vide minimum, maximum, and average heart rates during the monitoring
period, quantification of premature atrial and ventricular contractions, and
identification of brady and tachyarrhythmias (Fig. 6). In patients with more
rare symptoms and high suspicion for a potentially life-threatening arrhythmia,
an implantable loop recorder can be placed for up to several years.

Fig. 5. MRI of a 16-year old girl who presented with intermittent chest pain and dyspnea
2 weeks following a viral illness. (A) Short axis view depicting the left ventricle (LV) in cross
section, with area of late gadolinium enhancement (LGE), consistent with myocarditis involving
the posterior/inferior LV myocardium. (B) Four-chamber view demonstrating a small pericardial
effusion (PCE) consistent with pericardial inflammation. A, anterior; I, inferior; L, left; P, poste-
rior; R, right; RV, right ventricle; S, superior. (Image borrowed with permission from Wilson
King, MD, Associate Professor of Pediatrics, Saint Louis University.)

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180 DANON

Fig. 6. Holter monitor of a 16 year old female with palpitations, with sudden onset and termi-
nation, lasting from a few seconds up to a minute. The Holter captures a 5 beat run of ventric-
ular tachycardia (red arrows).

Exercise studies
In patients who have exertional symptoms, an exercise study can be done to
simulate a similar situation to what the patient was doing while experiencing
symptoms. An exercise treadmill study can be performed with EKG, blood
pressure, and pulse oximeter monitoring. This can detect exercise-induced
abnormal blood pressure response, ischemic changes, arterial desaturation,
and rhythm disturbances (Fig. 7). If there is concern for exercise-induced
asthma/bronchoconstriction, PFT can be performed before and after exercise
with the assessment of response to bronchodilator therapy. To obtain addi-
tional metabolic information, a cardiopulmonary exercise test is performed
with expiratory gas analysis through a mouthpiece analyzer. This is usually
reserved for patients with known underlying heart disease to differentiate car-
diac from noncardiac cause of symptoms or exercise limitations.

Fig. 7. Treadmill Exercise study in a 14 year old male with hypertrophic cardiomyopathy.
There are ischemic T-wave changes (red arrows) followed by an abrupt change in rhythm
(blue arrow) to Torsades de Pointes, a polymorphic ventricular tachycardia. (Image borrowed
with permission from Anjan Batra, MD, MBA, Professor of Pediatrics, University of California,
Irvine.)

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CHEST PAIN, PALPITATIONS, AND SYNCOPE 181

Cardiac catheterization and electrophysiologic studies


Attributable to availability of multiple noninvasive imaging modalities and
other diagnostic studies, cardiac catheterization is most often reserved for pa-
tients once a diagnosis is made, and there is indication for an interventional
procedure. It is usually performed with general anesthesia, and catheters are
inserted via the vein and artery, typically the femoral vessels. Pressure mea-
surements and blood gases are obtained from the cardiac chambers and vascu-
lature, and imaging is performed by the direct injection of contrast. Various
interventions may be performed for structural abnormalities, including device
closure of defects and abnormal vasculature, balloon valvuloplasty and angio-
plasty, stent placement, and transcatheter valve replacement.
Electrophysiologic studies are performed in a similar manner, using catheters
and specialized software to map the electrical activity of the heart. An
arrhythmia can be induced for diagnostic purposes. If an abnormal electrical
focus or pathway is identified, it can be ablated with radiofrequency or cryoa-
blation. If indicated, a permanent pacemaker or automatic implantable
cardioverter-defibrillator (AICD) can be placed (Fig. 8).

Genetic testing
Cardiovascular genetic testing is becoming more prevalent but should be used
with caution. Although it may identify a known pathogenic chromosomal ab-
normality or gene mutation, often results include a variant of unknown clinical
significance. It is most useful if there is a known family history of inheritable
genetic cardiac disease. In these situations, if the affected gene is identified,
then genetic testing can be performed in blood-related family members to assess
whether they have the same gene mutation. Otherwise, screening with

Fig. 8. Chest X-ray demonstrating placement of an automatic implantable cardioverter-


defibrillator (AICD) in a patient with hypertrophic cardiomyopathy following an episode of
aborted sudden cardiac arrest.

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182 DANON

diagnostic studies such as EKG and echocardiogram may need to be performed


at regular intervals. There are other scenarios where genetic testing may be use-
ful, but it should be reserved to situations with high likelihood of disease.
Appropriate pretesting and posttesting counseling should be performed in these
situations [17].

DISCUSSION
Symptoms that patients perceive to be cardiac in origin often cause significant
anxiety. This is exacerbated by the publicity following sudden cardiac death in
a child, despite these being very rare occurrences. Although chest pain, palpi-
tations, and syncope are usually benign, the challenge is to recognize and diag-
nose those associated with pathologic conditions that increase the risk for
sudden cardiac death. The two most common causes in the pediatric popula-
tion are hypertrophic cardiomyopathy and coronary artery abnormalities,
but there are multiple other causes that need to be ruled out when patients pre-
sent [1,2,18]. The history and physical examination often provide sufficient in-
formation, but additional testing is sometimes required to make a definitive
diagnosis or rule out pathology. Primary care and emergency physicians see
most of these patients and need to recognize the concerning characteristics of
these symptoms. If the symptoms are primarily exercise-related, patients
should discontinue physical activity until evaluation by a cardiologist. An
EKG helps in deciding whether to refer to a pediatric cardiologist, but a nega-
tive test does not rule out pathology.
Some patients may not present with specific complaints, but as part of an
annual physical or sports clearance. Although concern for cardiac disease is
sometimes focused on athletics, only 25% of cases of sudden cardiac death
are sports-related [2]. EKG is not part of the routine sports screening physical
examination. This is controversial in the United States but is mandated in some
countries such as Italy and Israel. Although there are some data that EKG
screening decreases the incidence of sudden death in athletes, there are also
data to the contrary [5,15,19,20]. Currently, the American Heart Association
and American Academy of Pediatrics (AAP) do not recommend including
EKG as routine sports screening for cardiovascular causes of sudden death.
In patients who have recent infection with SARS-CoV-2 with moderate or
severe symptoms, EKG should be performed before clearance to participate
in sports, and if abnormal, referred to cardiology before clearance [21]. In those
with asymptomatic or mild infection, EKG should be performed if they had
any cardiac-related symptoms. Patients should then follow a gradual return
to play protocol, and if they experience symptoms with exercise, discontinue
sports and have further evaluation. The reason for this is possible cardiac
inflammation or myocarditis related to SARS-CoV-2 infection. The AAP has
additional screening recommendations regarding previous SARS-CoV-2 infec-
tion, which may be found on their website [21].
Regardless of the cardiac diagnosis, the terminal rhythm resulting in sudden
cardiac arrest and death is pulseless ventricular tachycardia (VT) or ventricular

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CHEST PAIN, PALPITATIONS, AND SYNCOPE 183

fibrillation (VF) [1,22]. The definitive treatment is defibrillation, and survival


depends on how soon after the onset of the VT/VF it is performed [23]. Until
the time of defibrillation, it is important to perform good quality cardiopulmo-
nary resuscitation (CPR). Automated external defibrillators (AED) have
become much more readily available. They do not require medical expertise
and are present in many school, athletic complexes, airports, and other public
places. It is critical that when someone suddenly collapses, immediate action is
taken. Education of school and athletic personnel on appropriate CPR and defi-
brillation with an AED is crucial. In patients determined to be high risk for sud-
den cardiac arrest, an AICD can be placed.

SUMMARY
Chest pain, palpitations, and syncope are common complaints in pediatrics.
Clinicians need to identify patients at risk for sudden cardiac death. The
history and physical examination often provide adequate information, but
additional testing and referral to pediatric cardiology are sometimes indicated.

CLINICS CARE POINTS

 Sudden cardiac death is defined as an abrupt, unexpected death of cardiovascu-


lar cause with loss of consciousness within 1 hour of onset of symptoms.
 The 2 most common causes of sudden cardiac death in the pediatric population
are hypertrophic cardiomyopathy and coronary artery abnormalities.
 Exercise related symptoms including dyspnea, chest pain, palpitations, and syn-
cope warrant further diagnostic evaluation.
 Most pediatric chest pain is non-cardiac and includes musculoskeletal, gastrointes-
tinal, respiratory, and psychological etiologies.
 Wearable devices and phones are very helpful in documenting heart rate during
symptoms of palpitations.
 Palpitations that start and stop suddenly with documented heart rate >150 are
more likely to represent an arrhythmia.
 Neurally mediated causes of syncope often improve with increased hydration and
salt intake, adequate diet, improved sleeping habits, and daily exercise.
 Sinus arrhythmia is a common cause of irregular rhythm and represents a normal
finding of heart rate variation with the respiratory cycle.
 Genetic testing for cardiovascular diseases should be performed with caution and
appropriate counseling due to frequent results of unknown clinical significance.
 When evaluating cardiac symptoms, the history and physical examination often
provide sufficient information, but additional testing and referral to pediatric cardi-
ology are sometimes indicated.

DISCLOSURES
The author does not have any commercial or financial conflicts of interest.

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References
[1] Zipes DP, Wellens HJ. Sudden cardiac death. Circulation 1998;98(21):2334–51.
[2] Gajewski KK, Saul JP. Sudden cardiac death in children and adolescents (excluding Sudden
Infant Death Syndrome). Ann Pediatr Cardiol 2010;3(2):107–12.
[3] Kakavand B. Dizziness, Syncope, and Autonomic Dysfunction in Children. Prog Pediatr
Cardiol 2022;65:101512.
[4] Wackel P, Cannon B. Heart Rate and Rhythm Disorders. Pediatr Rev 2017;38(6):243–53.
[5] Maron BJ, Friedman RA, Kligfield P, et al. Assessment of the 12-lead electrocardiogram as a
screening test for detection of cardiovascular disease in healthy general populations of
young people (12-25 years of age): a scientific statement from the American Heart Associ-
ation and the American College of Cardiology. J Am Coll Cardiol 2014;64(14):
1479–514.
[6] Maron BJ, Thompson PD, Ackerman MJ, et al. Recommendations and Considerations
Related to Preparticipation Screening for Cardiovascular Abnormalities in Competitive Ath-
letes: 2007 Update: A Scientific Statement From the American Heart Association Council on
Nutrition, Physical Activity, and Metabolism: endorsed by the American College of Cardi-
ology Foundation. Circulation 2007;115(12):1643–2455.
[7] Barbut G, Joshua PN. Pediatric Chest Pain. Pediatr Rev 2020;41(9):469–80.
[8] Schwartz BN, Harahsheh AS, Krishnan A, et al. Cardiac Effects of COVID-19 Infection, MIS-
C, and the Vaccine in Infants and Children: What Is Known and Future Implications. Am J
Perinatol 2022;39(S 01):S1–6.
[9] Mamishi A, Olfat M, Pourakbari B, et al. Multisystem inflammatory syndrome associated
with SARS-CoV-2 infection in children: update and new insights from the second report of
an Iranian referral hospital. Epidemiol Infect 2022;150:e179.
[10] Johnson JN, Driscoll DJ. Chest pain in children and adolescents. In: Allen H, Shaddy R,
Penny D, et al, editors. Moss and adams heart disease in infants, children, and adolescents.
9th edition. Riverwoods, IL: Wolters Kluwer; 2016. p. 1627–31.
[11] Flynn JT, Kaelber DC, Baker-Smith CM, et al. Clinical Practice Guideline for Screening and
Management of High Blood Pressure in Children and Adolescents. Pediatrics 2017;140(3):
e20171904.
[12] Freeman R, Wieling W, Axelrod F, et al. Consensus statement on the definition of orthostatic
hypotension, neurally mediated syncope and the postural tachycardia syndrome. Auton
Neurosci 2011;161(1–2):46–8.
[13] Regalado ES, Morris SA, Braverman AC, et al. Comparative Risks of Initial Aortic Events
Associated With Genetic Thoracic Aortic Disease. J Am Coll Cardiol 2022;80(9):857–69.
[14] Holmes KW, Markwardt S, Eagle KA, et al. Cardiovascular Outcomes in Aortopathy Gen-
TAC Registry of Genetically Triggered Aortic Aneurysms and Related Conditions. J Am Coll
Cardiol 2022;79(21):2069–81.
[15] Williams E, Pelto H, Toresdahl B, et al. Performance of the American Heart Association
(AHA) 14-Point Evaluation Versus Electrocardiography for the Cardiovascular Screening
of High School Athletes: A Prospective Study. J Am Heart Assoc 2019;8(14):e012235.
[16] Chiu CC, Hamilton RM, Gow RM, et al. Evaluation of computerized interpretation of the pe-
diatric electrocardiogram. J Electrocardiol 2007;40(2):139–43.
[17] Landstrom AP, Kim JJ, Gelb BD, et al. Genetic Testing for Heritable Cardiovascular Diseases
in Pediatric Patients: A Scientific Statement From the American Heart Association. Circ Ge-
nom Precis Med 2021;14(5):e000086.
[18] Maron BJ, Shirani J, Poliac LC, et al. Sudden death in young competitive athletes. Clinical,
demographic, and pathological profiles. JAMA 1996;276(3):199–204.
[19] Corrado D, Basso C, Pavei A, et al. Trends in sudden cardiovascular death in young compet-
itive athletes after implementation of a preparticipation screening program. JAMA
2006;296(13):1593–601.

Downloaded for Román Wagner Thomas ESTELI CHÁVEZ (rwtestelic@yopmail.com) at


Thammasat University from ClinicalKey.com by Elsevier on August 22, 2023. For personal use
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CHEST PAIN, PALPITATIONS, AND SYNCOPE 185

[20] Steinvil A, Chundadze T, Zeltser D, et al. Mandatory electrocardiographic screening of ath-


letes to reduce their risk for sudden death proven fact or wishful thinking? J Am Coll Cardiol
2011;57(11):1291–6.
[21] COVID-19 Interim Guidance. Return to Sports and Physical Activity. In: American Academy
of pediatrics. 2022 Avalable at: https://www.aap.org/en/pages/2019-novel-coronavi-
rus-covid-19-infections/clinical-guidance/covid-19-interim-guidance-return-to-sports/
2022. Accessed 10 October, 2022.
[22] Gadage SN. T-wave alternans in long QT syndrome. Ann Pediatr Cardiol 2018;11(2):
219–21.
[23] Fukuda T, Ohashi-Fukuda N, Kobayashi H, et al. Public access defibrillation and outcomes
after pediatric out-of-hospital cardiac arrest. Resuscitation 2017;111:1–7.

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