Doctor examining girlChildren with chest pain commonly present to the emergency department. Both the child and family members may think their symptoms are due to a serious illness. Among adolescents seen for their chest pain, more than 50% thought they were having a heart attack or that they had cancer.1 In reality, only 6% of pediatric chest pain has a cardiac etiology.2 Nonetheless, extensive and costly emergency department (ED) evaluations are common and there is wide practice variation.3

But prior to reassuring your patient, what can you do to reassure yourself that your patient doesn’t need a more extensive workup? What would make you suspicious for cardiac causes of pediatric chest pain?

Sherlock Holmes-ing for historical clues

While chest pain in high-risk adults necessitates a standardized evaluation, not all chest pain in children requires an automatic workup. Good history-taking skills and physical exam can help you decide which patients will need x-rays, EKGs, or labs. Ask about a history or family history of Kawasaki disease, asthma, sickle cell disease, diabetes, or connective tissue disorders, such as Marfan, Turner, and Ehlers-Danlos syndromes. Probing for family history of unexplained or sudden death, drowning, or heart disease may also help to identify children at risk for arrhythmia or structural heart disease.1 Historical clues such as the event surrounding the onset of chest pain, pain location, or presence of radiation can be helpful. The presence of exertional chest pain or syncope may warrant further cardiac evaluation.

Physical exam

Fever and tachypnea may suggest infectious etiologies such as pneumonia or pericarditis. Chest pain that worsens with laying down may also indicate pericarditis with or without effusion. Hypotension and jugular venous distension raises concern for cardiomyopathy. On the other hand, reproducible chest tenderness makes a cardiac etiology less likely.

Distant heart sounds or a friction rub on cardiac examination may indicate effusion or pericarditis. Patients with coarctation of the aorta have weak femoral pulses and right arm hypertension. Not all murmurs require referral. Still’s murmurs are common and need no work up. They sound vibratory or musical, and decrease in intensity when the patient stands. To listen to what these sounds like, see below or click here.

Pathological murmurs are loud, harsh, or diastolic. Left ventricular outflow obstructions will have a harsh, systolic murmur that radiates to the neck. The murmur of hypertrophic obstructive cardiomyopathy (HOCM) gets louder when the patient stands. For an example of HOCM’s harsh murmur, click here.

Break down the differential into non-cardiac and cardiac causes

Reddy et al. has a useful review article in Pediatrics in Review, which provides a good summary of how to approach pediatric chest pain. Non-cardiac causes include musculoskeletal, pulmonary, gastrointestinal or psychiatric causes (Table 1).2 Common musculoskeletal considerations includes muscle strain or trauma, costochondritis, Tietze syndrome (localized inflammation of the costochondral, costosternal or sternoclavicular joint), and idiopathic chest wall pain. Respiratory causes such as asthma can present with chest pain without any audible wheezing. Patients with respiratory infections such as pneumonia may also have chest pain. Common gastrointestinal causes of chest pain include gastroesophageal reflux disease (GERD), peptic ulcer disease, and cholecystitis.

Musculoskeletal
Costochondritis/costosternal syndrome
Tietze syndrome
Nonspecific or idiopathic chest wall pain
Slipping rib syndrome
Trauma and muscle-overuse injury
Xiphoid pain
Sickle cell vaso-occlusive crisis
Pulmonary or Airway-Related
Asthma
Bronchitis
Pleuritis
Pneumonia
Pneumothorax
Pulmonary embolism
Acute chest syndrome
Gastrointestinal
Gastroesophageal reflux disease
Esophageal spasm
Peptic ulcer disease
Drug-induced esophagitis/gastritis
Cholecystitis
Miscellaneous
Panic disorder
Hyperventilation
Breast-related conditions
Herpes zoster
Spinal cord or nerve root compression
Table 1. Non-cardiac causes of pediatric chest pain (adapted from Reddy et al.)

Cardiac causes of chest pain

Infectious or inflammatory causes

Children with pericarditis, myocarditis, or endocarditis may present with sharp retrosternal chest pain that worsens with deep breaths or when lying flat.2,3 Their pain may improve when leaning forward. Patients may have symptoms of infection (eg. rheumatic fever, coxsackie, ECHO or influenza) or have a history of inflammatory diseases, such as lupus. On exam, these patients may have a friction rub, fever, heart murmur, or shortness of breath, along with flu-like symptoms. Embolization and dissemination of infected vegetation will lead to various different organ injuries and their associated symptoms.4

Coronary artery and structural abnormalities

Coronary artery and structural abnormalities can present in older children as anginal symptoms. Beware that with younger children they may present as colicky or irritable. Coronary anomalies may precipitate myocardial ischemia or sudden cardiac death. Children with a history of cardiac surgery or transplant are at risk of myocardial ischemia or transplantation rejection leading to accelerated coronary artery vasculopathy or tacchyarrhythmias. In addition, heart surgeries that potentially affect the coronary arteries (for example correction of transposition of the great arteries) increase the risk of developing coronary artery stenoses. Patients with a history of Kawasaki disease and associated coronary artery aneurysms are at high risk of coronary artery stenosis, rupture, or thrombosis. The risk of coronary aneurysm is highest in the first 5 weeks after Kawasaki diagnosis.5 Patients that have a familial history of hypercholesterolemia may present in their early 20s with coronary artery disease, and therefore, should have follow-up with their pediatrician to determine if more testing is necessary. Intrinsic or structural abnormalities, such as septal defects, left-to-right shunts, or cardiomyopathies may present as chest pain, but are usually associated with palpitations, fatigue, exercise intolerance, dizziness, or exertional syncope.

Arrhythmogenic causes

Premature ventricular complexes (PVCs) may elicit chest pain in patients. However, for many children with arrhythmias, they may not present with classic complaints of chest pain or be able to describe “palpitations.” In children under 12 years, the most common cause of SVT is an accessory atrioventricular pathway, whereas in teenagers, it tends to be atrioventricular node re-entry tachycardia. Patients with congenital QT syndrome may present in the pre-teens or teenage years with seizure or syncope.4

Patients that you suspect may have cardiac cause of their chest pain will warrant further evaluation.  

Inflammatory: Pericarditis, myocarditis
Infective Viruses, bacteria
Non-infective SLE, Crohn disease, postpericardiotomy syndrome
Coronary artery abnormalities
Congenital ALCAPA, ALCA from right coronary sinus, coronary fistula
Acquired Kawasaki disease, post-surgical, post-transplant coronary vasculopathy, familial hypercholesterolemia
Increased myocardial demand or decreased supply
Cardiomyopathy Dilated or hypertrophic
LVOT obstruction Aortic, supra-aortic, or supra-valvar aortic stenosis
Arrhythmias
Drugs
 Cocaine
Sympathomimetic overdose
Miscellaneous
Aortic dissection
Rupture of aortic aneurysm
Mitral valve prolapse
Cardiac device/stent complications
Atrial myxoma
Pulmonary hypertension
Connective tissue disease
Table 2. Cardiac causes of chest pain (adapted from Reddy et al.)

Further evaluation

Chest x-ray: Look for evidence of cardiomegaly, pulmonary vascular congestion, pneumothorax, or fractures.

Troponin: Routine troponin levels for all pediatric chest pain is not recommended. In studies, troponins were normal in 99% of children with chest pain who also had normal EKGs and were afebrile. In selected children with chest pain who also had abnormal EKG findings or a fever, obtaining elevated troponin had diagnostic utility for diagnosing myopericarditis. In addition, troponin may be useful in children with chest pain associated with chest trauma, overdose, poisoning, or significant cardiac pathology.4

EKG: ST segment changes may indicate ischemia or myocarditis. Other pediatric EKG findings associated with chest pain are absent p-waves (SVT), delta waves and shortened PR intervals (Wolff Parkinson White), a long QT interval, deep or wide Q-waves (MI or hypertrophy). Large R waves in V6 and deep S waves in V1 indicate left ventricular hypertrophy while the opposite (large R waves in V1 and deep S waves in V6) indicate right ventricular hypertrophy. Intervals and voltages should be compared to age-appropriate normal values.

For an excellent interactive review of pedatric EKGs, check out these videos on:

Patients awaiting a pediatric cardiologist referral, such as for an echocardiography or cardiac catheterization, should be advised to restrict their physical activity.

Bottom line

  1. Most episodes of chest pain in kids have non-cardiac causes.
  2. Good history-taking and physical exam can help guide what type of diagnostics are needed, if any.
  3. Be suspicious in children that are colicky, have palpitations, lightheadedness, syncope, seizure, or exertional symptoms. These children may have an underlying cardiac cause to their chest pain.
  4. Troponins are rarely useful in afebrile pediatric patients presenting with chest pain and a normal EKG.
  5. To learn more about mumurs, take a listen to www.easyauscultation.com.
  6. Check out PEM Academy which has all sorts of case-based pediatric emergency medicine learning, but it also has great educational videos on Pediatric EKGs.

1.
Thull-Freedman J. Evaluation of chest pain in the pediatric patient. Med Clin North Am. 2010;94(2):327-347. [PubMed]
2.
Reddy S, Singh H. Chest pain in children and adolescents. Pediatr Rev. 2010;31(1):e1-9. [PubMed]
3.
Friedman K, Kane D, Rathod R, et al. Management of pediatric chest pain using a standardized assessment and management plan. Pediatrics. 2011;128(2):239-245. [PubMed]
4.
Sharieff G, Wylie T. Pediatric cardiac disorders. J Emerg Med. 2004;26(1):65-79. [PubMed]
5.
McCrindle B, Li J, Minich L, et al. Coronary artery involvement in children with Kawasaki disease: risk factors from analysis of serial normalized measurements. Circulation. 2007;116(2):174-179. [PubMed]
Delphine Huang, MD

Delphine Huang, MD

Resident Department of Emegency Medicine (EM) UCSF-San Francisco General Hospital EM Residency Program
Sonny Tat, MD MPH

Sonny Tat, MD MPH

Assistant Professor
Pediatric Emergency Medicine
University of California, San Francisco