Professional Documents
Culture Documents
ADVANCES IN PEDIATRICS
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,
https://doi.org/10.1016/j.yapd.2023.04.003
0065-3101/23/ª 2023 Elsevier Inc. All rights reserved.
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
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.
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.
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.
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.
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.)
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.
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.)
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.)
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.)
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
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
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.
DISCLOSURES
The author does not have any commercial or financial conflicts of interest.
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.