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Nontraumatic chest pain in children and adolescents: Approach and initial management

Nontraumatic chest pain in children and adolescents: Approach and initial management
Literature review current through: May 2024.
This topic last updated: May 08, 2024.

INTRODUCTION — Nontraumatic chest pain is a common symptom in children and adolescents and is a frequent complaint in patients seeking primary, emergency, or subspecialty care [1-4]. Although the etiology is benign in most cases, this symptom may lead to school absences, restriction of activities and causes considerable anxiety in patients and their families or caregivers. A thorough history and physical examination usually can determine the cause and identify patients who require acute intervention and those who can be managed with reassurance and continued follow-up. Laboratory testing is necessary only in a small number of patients [5]. In the absence of associated symptoms of illness, positive findings on physical examination related to the cardiac or respiratory systems, or symptoms during exertion, a serious organic cause is unlikely.

This topic will discuss the approach to nontraumatic chest pain in children and adolescents. The causes of nontraumatic chest pain in children and adolescents, pediatric thoracic trauma, and the emergent evaluation of respiratory distress in children and adolescents, with or without chest pain, is discussed separately:

(See "Causes of nontraumatic chest pain in children and adolescents".)

(See "Overview of intrathoracic injuries in children".)

(See "Thoracic trauma in children: Initial stabilization and evaluation".)

(See "Chest wall injuries after blunt trauma in children".)

(See "Pulmonary contusion in children".)

(See "Acute respiratory distress in children: Emergency evaluation and initial stabilization".)

EVALUATION — The clinician should perform a careful history and physical examination in all children with nontraumatic chest pain with the goal of identifying those patients who warrant further diagnostic studies or referral.

History — Most children with nontraumatic chest pain appear well during acute evaluation. Thus, the history is of utmost importance for identifying serious underlying causes of pediatric chest pain.

Findings of life-threatening causes — The following symptoms are associated with potentially life-threatening cardiac or pulmonary causes of pediatric chest pain (table 1) [3] (see "Causes of nontraumatic chest pain in children and adolescents", section on 'Life-threatening conditions'):

Pain description as follows:

Classic anginal pain (ie, substernal, crushing, with radiation down the left arm or up into the jaw and associated with vomiting, diaphoresis, altered mental status, or dyspnea)

Severe, tearing type pain often radiating to the back (aortic dissection)

Pleuritic pain with difficulty breathing (pneumonia, pneumothorax, pulmonary embolus, or in patients with sickle cell disease, acute chest syndrome)

Exertional pain or exercise intolerance due to tachypnea or fatigue caused by limitations in myocardial oxygen delivery (myocardial ischemia, coronary anomalies, cardiomyopathy)

Sharp, retrosternal pain exacerbated by lying down, sometimes radiating to the left shoulder, and often associated with fever (pericarditis)

Pain precipitated by the use of cocaine, amphetamines, bath salts, synthetic cannabinoids, marijuana or other vasoactive drugs (eg, cough and cold preparations) suggesting variant angina

Symptoms occurring with pain as follows:

Exertional syncope or dizziness (coronary anomalies, left ventricular outflow obstruction, cardiomyopathy)

Palpitations

Difficulty breathing

Fever and heart failure symptoms (eg, dyspnea on exertion, orthopnea, or in infants, tachypnea with feedings) suggestive of myocarditis

Concerning past medical history such as:

Congenital or acquired heart disease, Kawasaki disease, or sickle cell anemia

Receipt of an mRNA coronavirus disease 2019 (COVID-19) vaccine within the preceding 30 days (see "COVID-19: Vaccines", section on 'Myocarditis')

Conditions that predispose to pericarditis, for example, rheumatologic disease, malignancy, recent cardiac surgery, mediastinal radiation, renal failure or infections such as tuberculosis, human immunodeficiency virus

Risk factors for pulmonary embolism including immobility, oral contraceptive use, pregnancy termination, ventriculoatrial shunts for hydrocephalus, in-dwelling central venous catheters, solid tumors, heart disease, hypercoagulable states, and obesity

Previous aortic dissection, particularly in a patient known to have Marfan, Loeys-Dietz, type IV Ehlers-Danlos, or Turner syndrome

Family history of:

Hypertrophic cardiomyopathy or of sudden death in first-degree relatives (eg, parents or siblings) younger than 50 years of age

Marfan, Loeys-Dietz, type IV Ehlers-Danlos, or Turner syndromes because of predisposition for aortic root dissection

Inherited hypercoagulable state (factor V Leyden, protein C or protein S deficiency, and other conditions)

Other important features — When history does not suggest a life-threatening cause, an accurate description of the chest pain can help establish a specific diagnosis as follows (table 2) [3,6-8]:

Temporal elements – The duration and time course of the onset of chest pain may be a useful distinguishing feature. Chronic pain is unlikely to have a serious underlying cause and often is musculoskeletal, psychogenic, or idiopathic.

Acute pain is more likely to be caused by a medical condition. As examples, pain caused by pulmonary conditions (eg, asthma or pneumothorax) or vascular events (eg, aortic dissection or acute pulmonary embolism) typically has an abrupt onset. However, ischemic myocardial pain, which is unusual in children and is described in greater detail above, may have a gradual onset with increasing intensity over time.

Quality – A description of the quality of the pain may be helpful. The pain associated with costochondritis typically is described as midsternal in location and sharp in quality with minimal radiation. It occurs mainly at rest and lasts for seconds to a few minutes. The pain may increase in intensity with deep inspiration because of stretching of the costochondral junctions or muscle fibers.

Patients with slipping rib syndrome may describe "something slipping or giving away," "a popping sensation," or "hearing a clicking sound" [9]. In that condition, pain frequently occurs with bending over or deep breathing [10].

Location – Pain that localizes to a small area on the chest more likely is of chest wall or pleural origin rather than visceral. Ischemic pain is a diffuse discomfort that may be difficult to localize.

Radiation usually is associated with causes of pain that are uncommon in children. As an example, the pain of myocardial ischemia may radiate to the neck, throat, lower jaw, teeth, upper extremity, or shoulder. Other unusual causes include acute cholecystitis, which can present with right shoulder pain (although concomitant right upper quadrant or epigastric pain is more typical), aortic dissection, which may be associated with chest pain that radiates between the scapulae, or pericarditis that can radiate to the left shoulder.

Precipitating factors – The patient should be asked about factors that induce or make the pain worse:

Deep breathing may exacerbate chest pain of musculoskeletal origin.

Pain made worse by swallowing likely is of esophageal origin. Discomfort that occurs with eating also may suggest upper gastrointestinal disease. History of a choking spell or witnessed foreign body ingestion, especially in a preschool child warrants evaluation for an esophageal foreign body.

Chest discomfort provoked by exertion often has a cardiac or respiratory cause.

Pleuritic chest pain is worsened by inspiration.

Chest pain that increases when lying supine and decreases when sitting suggests pericarditis.

Pain associated with coronary artery anomalies typically occurs with exertion.

Associated symptoms – Associated symptoms may help determine etiology.

Fever, especially when associated with tachypnea or cough, may suggest a respiratory infection. Fever is also present in patients with pericarditis, myocarditis, Kawasaki disease, or multisystem inflammatory syndrome in children (MIS-C). (See "Clinical manifestations and diagnosis of myocarditis in children" and "Kawasaki disease: Clinical features and diagnosis" and "COVID-19: Multisystem inflammatory syndrome in children (MIS-C) clinical features, evaluation, and diagnosis".)

Dyspnea or cough may indicate pulmonary disorders, including pathology of the airways, lung parenchyma, or pulmonary vasculature, or be a sign of cardiac disease. In addition to chest pain, for example, patients with pulmonary embolism can develop dyspnea, hypoxemia, apprehension, cough, and diaphoresis [11]. Patients with myocarditis may have dyspnea and fatigue. (See "Clinical manifestations and diagnosis of myocarditis in children".)

Vomiting or regurgitation, painful swallowing, or heartburn associated with eating suggest gastrointestinal disease, such as gastroesophageal reflux and esophagitis.

Recurrent somatic complaints, including headache or abdominal or extremity pain, occur in most children with psychogenic chest pain [12,13]. Approximately one-third have significant sleep disturbances [12].

Lightheadedness or paresthesias frequently accompany chest pain resulting from hyperventilation.

Exertional syncope or palpitations suggest an underlying cardiac disorder. (See "Emergency evaluation of syncope in children and adolescents".)

Physical examination — Children and adolescents with chest pain are typically in no acute distress or have minimal discomfort during examination.

Patients with acute onset of chest pain and who exhibit respiratory distress or hemodynamic instability require immediate management according to the principles of Pediatric Advanced Life Support. This should be initiated while the evaluation proceeds. (See 'Unstable' below.)

A thorough physical examination should be performed in all patients. Important vital sign changes include [3,6]:

Fever (associated with cardiac or pulmonary disease such as pericarditis, myocarditis, pneumonia, or a rheumatologic condition)

Tachycardia for age (table 3) (present in several common and life-threatening causes of pediatric chest pain and warrants evaluation of the patient’s rhythm and cardiac function)

Tachypnea for age (table 3) (seen in patients with a variety of conditions but raises concerns for asthma, pneumonia, spontaneous pneumothorax, pulmonary embolism, pulmonary congestion from cardiac causes, and hyperventilation syndrome)

Hypertension (frequently present in patients with aortic dissection)

Hypotension (associated with serious cardiac, pulmonary, and infectious causes of chest pain)

Narrow pulse pressure or pulsus paradoxus >10 mmHg (present in patients with a large pericardial effusion associated with cardiac tamponade)

Hypoxia (eg, abnormal pulse oximetry; the threshold varies based on several factors, but, for normal individuals at sea level, many experts consider a resting oxygen saturation ≤95 percent as abnormal. Trends in oxygen saturation and the underlying disease process are important for interpretation.) (See "Pulse oximetry", section on 'Optimal oxygen saturation'.)

General appearance may indicate the following [3,6,8]:

Presence of genetic conditions that are associated with congenital heart disease (eg, Turner syndrome) or aortic dissection (eg, Marfan (table 4) or Ehlers-Danlos syndrome). (See "Clinical manifestations and diagnosis of Ehlers-Danlos syndromes", section on 'Clinical manifestations and diagnosis' and "Clinical manifestations and diagnosis of Turner syndrome", section on 'Clinical manifestations'.)

Signs of chronic disease such as weight loss, fatigue, or pallor suggesting an underlying condition such as systemic lupus erythematosus, lymphoma, or a solid tumor.

Tetany or carpopedal spasm indicating hyperventilation syndrome. (See "Hyperventilation syndrome in adults", section on 'Clinical presentation'.)

Drooling associated with an esophageal foreign body

Next, the clinician should focus on the chest, lungs, and heart as follows [3,6,8]:

Chest wall – Chest wall deformity, such as pectus excavatum or carinatum (although uncommonly causes of chest pain), asymmetry of breathing (pneumothorax), rapid, shallow breathing with normal oxygenation (hyperventilation) or asymmetry of the breasts (gynecomastia) are important findings on inspection.

Chest wall tenderness indicates a musculoskeletal cause of pain, typically costochondritis. Bruises associated with trauma sometimes are seen. However, serious underlying thoracic injury can occur without obvious chest wall bruising. (See "Overview of intrathoracic injuries in children" and "Pulmonary contusion in children".)

Examination should also include palpation of the costochondral junctions, the insertion site of the pectoralis major muscle group (by grasping the head of the muscle between the examiner's fingers and thumb), the inframammary area, and other regions of the chest where pain is reported. In patients with costochondritis, involvement of the costochondral junctions often is asymmetric, with the left side more frequently affected. The diagnosis is confirmed when palpation reproduces the discomfort. Provocative maneuvers for costochondritis include horizontal arm traction (figure 1) and the “crowing rooster” maneuver (figure 2). (See "Causes of nontraumatic chest pain in children and adolescents", section on 'Musculoskeletal'.)

Diagnosis of slipping rib syndrome can be confirmed by the "hooking" maneuver in which the examiner's fingers are curled around the lower costal margin. Lifting anteriorly will elicit a click and reproduce pain (figure 3). (See "Causes of nontraumatic chest pain in children and adolescents", section on 'Musculoskeletal'.)

Tenderness on palpation of breast tissue often can be elicited in patients who complain of breast pain. Tender subareolar masses may also be palpable. (See "Breast disorders in children and adolescents", section on 'Breast pain'.)

Lungs – Signs of a respiratory etiology may include tachypnea and respiratory distress. Patients with respiratory distress warrant urgent assessment of airway and breathing and administration of supplemental oxygen. Airway management in patients with signs of impending respiratory failure should be initiated prior to full evaluation. (See "Acute respiratory distress in children: Emergency evaluation and initial stabilization", section on 'Rapid assessment'.)

In addition to lung pathology, tachypnea with or without wheezing can also be an important sign of heart failure in children with myocarditis, dilated cardiomyopathy, or other cardiac causes of chest pain. (See "Heart failure in children: Etiology, clinical manifestations, and diagnosis", section on 'Clinical manifestations'.)

Diminished breath sounds over affected areas of the lung raises suspicion for pneumothorax. Rales or tubular breath sounds suggest pneumonia whereas wheezing may be audible in patients with asthma. Pneumomediastinum or esophageal rupture may produce subcutaneous emphysema that is detected as crepitus on palpation of the supraclavicular or neck region. (See "Causes of nontraumatic chest pain in children and adolescents", section on 'Spontaneous pneumothorax' and "Causes of nontraumatic chest pain in children and adolescents", section on 'Respiratory'.)

Hyperventilation, associated with chest pain of psychogenic origin, may be present at the time of the examination. Patients will display light, shallow breathing with normal lung sounds and oxygenation. If the patient is not actively hyperventilating, it may be possible to reproduce the chest pain by asking the patient to hyperventilate. However, hyperventilation for as long as 20 minutes may be required to reproduce symptoms [14].

In patients with atypical angina due to coronary vasospasm, hyperventilation for six minutes can frequently reproduce symptoms [15].

Heart – A cardiac cause of chest pain is identified by abnormal heart sounds on auscultation (eg, cardiac murmur, gallop, muffled heart sounds, or a pericardial friction rub) or by an abnormal pulse or blood pressure (table 1). Any new heart murmur or abnormal heart sound in association with chest pain should prompt additional evaluation and pediatric cardiology consultation. (See 'Ancillary studies' below and 'Indications for pediatric subspecialty consultation or referral' below.)

Findings associated with specific cardiac causes of chest pain include:

Hypertrophic cardiomyopathy (HCM) – Patients with HCM may develop several types of systolic murmurs, but the two most common are related to left ventricular outflow tract (LVOT) obstruction and mitral regurgitation as follows (see "Hypertrophic cardiomyopathy: Clinical manifestations, diagnosis, and evaluation", section on 'Systolic murmurs'):

-Significant LVOT obstruction results in a harsh crescendo-decrescendo systolic murmur that begins slightly after S1 and is heard best at the apex and lower left sternal border. The murmur may radiate to the axilla and base but usually not into the neck. It increases with a Valsalva maneuver.

-Centrally directed mitral regurgitation, usually associated with primary mitral valve pathology, classically results in a holosystolic murmur heard loudest at the apex which radiates to the axilla.

Dilated cardiomyopathy – Pediatric patients with dilated cardiomyopathy may display findings of heart failure that vary by age but include tachycardia, poor perfusion, an S3 gallop, tachypnea, wheezing, hepatomegaly, jugular venous distension, dyspnea on exertion, and peripheral edema [16]. (See "Heart failure in children: Etiology, clinical manifestations, and diagnosis", section on 'Physical examination'.)

Coronary ischemia – Myocardial ischemia may result in tachycardia, increased blood pressure, new murmur consistent with mitral valve regurgitation, paradoxical splitting of the second heart sound if left bundle branch block is present, or a third or fourth heart sound. Some patients have none of these findings. (See "Approach to the patient with suspected angina pectoris", section on 'Physical examination'.)

Left ventricular outflow obstruction – Signs of left ventricular outflow obstruction include a systolic ejection murmur at the right upper sternal border and occasionally along the left sternal border. Coarctation of the aorta is associated with elevated blood pressure in the arms and a lower blood pressure in the legs, and frequently with a systolic murmur on the back between the scapulae. If the coarctation is long-standing (present for more than five to seven years), collateral vessels may form that connect the upper and lower portions of the aorta; these vessels create a continuous murmur over the lateral aspect of the ribs. (See "Clinical manifestations and diagnosis of coarctation of the aorta".)

Arrhythmia – An irregular heart rate associated with chest pain increases the likelihood of an underlying arrhythmia. Very rapid tachycardia for age (180 to 240 beats per minute in older children and adolescents) suggests supraventricular tachycardia, including Wolff-Parkinson-White (WPW) syndrome. Exposure to a variety of sympathomimetic agents, including cocaine, amphetamines, bath salts, and synthetic cannabinoids may cause sinus tachycardia, hypertension, and anxiety. (See "Irregular heart rhythm (arrhythmias) in children", section on 'Symptomatic child' and "Clinical features and diagnosis of supraventricular tachycardia (SVT) in children", section on 'Clinical features'.)

Pericarditis – In patients with pericarditis, pain increases when manual pressure is applied to the sternal region. The pain typically improves with sitting up and leaning forward. Signs of pericarditis depend upon the size of the pericardial effusion. Patients with a small effusion typically have an audible pericardial friction rub, caused by rubbing together of the inflamed parietal and visceral pericardial surfaces. The rub often is continuous in systole and diastole. It is easier to hear with the diaphragm of the stethoscope when the patient is sitting and leaning forward.

A rub will not be heard if the effusion is large because the two pericardial surfaces of the pericardium are not in contact with each other. A large effusion may result in cardiac tamponade, manifested by a narrow pulse pressure, elevated pulsus paradoxus (>10 mmHg), elevated jugular venous pressure, distant heart sounds, hepatomegaly, ascites, and peripheral edema.

Myocarditis – Myocarditis may on occasion present with chest pain. Signs of myocarditis include tachycardia, diminished heart sounds, a gallop rhythm, and a murmur of mitral regurgitation, usually accompanied by fever. Patients often complain of dyspnea and fatigue and may display tachypnea on examination. Tachycardia out of proportion to age and poor perfusion can be findings of myocarditis in children with an otherwise normal examination. (See "Clinical manifestations and diagnosis of myocarditis in children".)

Pulmonary hypertension (pulmonary embolism) – Patients with pulmonary hypertension caused by a pulmonary embolus typically have a right ventricular heave and a single loud S2. They may have a murmur consistent with tricuspid or pulmonary regurgitation.

Additional findings that suggest specific diagnoses include [6,8]:

Epigastric abdominal tenderness associated with gastritis or, less commonly, pancreatitis

Rashes (eg, evanescent salmon pink rash (picture 1) or malar rash (picture 2)) characteristic of specific collagen vascular diseases

Arthritis arising from underlying collagen vascular disease (eg, juvenile rheumatoid arthritis or systemic lupus erythematosus)

Posterior oral ulcers and rash on the palms and soles in associated with pleurodynia in patients with enterovirus infection (coxsackievirus B)

Ancillary studies — Most children with chest pain have no history suggestive of life-threatening conditions (table 1) and either a normal physical examination or findings consistent with a musculoskeletal etiology. Further investigations are not needed in patients for whom a clear etiology, other than cardiac disease, can be established [3,6,8]. Furthermore, in children with findings that indicate gastroesophageal reflux disease or medication-induced esophagitis, presumptive treatment prior to testing is appropriate [8]. (See "Management of gastroesophageal reflux disease in children and adolescents", section on 'Lifestyle changes' and "Management of gastroesophageal reflux disease in children and adolescents", section on 'Pharmacotherapy' and "Pill esophagitis", section on 'Management'.)

Diagnostic studies may help establish a diagnosis in patients with associated symptoms that suggest pulmonary or cardiac conditions or an esophageal foreign body [3,6]. Although cardiac causes of chest pain are uncommon in children, patients with clinical manifestations that are concerning for life-threatening cardiac conditions warrant prompt consultation with a pediatric cardiologist (table 1). (See 'Indications for pediatric subspecialty consultation or referral' below and "Causes of nontraumatic chest pain in children and adolescents", section on 'Cardiac disease'.)

Bedside ultrasonography — When performed by properly trained and experienced clinicians, bedside ultrasound can rapidly confirm the presence of pneumothoraces and pericardial effusions and guide emergent intervention (eg, chest tube thoracostomy or pericardiocentesis (figure 4)) in unstable patients. (See "Emergency pericardiocentesis", section on 'Ultrasound-guided pericardiocentesis technique' and "Bedside pleural ultrasonography: Equipment, technique, and the identification of pleural effusion and pneumothorax".)

Electrocardiogram — An electrocardiogram (ECG) should be obtained if cardiac disease is suspected based upon history or physical examination (table 1) and when a noncardiac etiology (eg, costochondritis, pneumonia, gastroesophageal reflux, pill esophagitis, or esophageal foreign body) cannot be established.

In patients with a rapid pulse rate or palpitations, an ECG can identify the type of arrhythmia (algorithm 1). However, if the arrhythmia is intermittent and the initial ECG is normal, referral to a pediatric cardiologist for a Holter monitor or event monitor recording may be needed.

Other ECG abnormalities can help with diagnosis and indicate the need for further testing as follows [3]:

Patients with hypertrophic cardiomyopathy may have evidence of left ventricular hypertrophy or strain. Formal exercise tolerance testing may be needed to assess the development of arrhythmia, left ventricular outflow tract obstruction, or ischemia during exertion.

In pericarditis with effusion, the ECG changes during the clinical course. Generalized ST segment elevation involving limb and precordial leads is seen in the initial 10 to 14 days (waveform 1). This is followed by T wave flattening or inversion that can persist for an additional two weeks. Voltages will be reduced in amplitude if the effusion is large. Abnormal Q waves typically are not seen in pericarditis. ST-T wave abnormalities occur in myocarditis. (See "Clinical manifestations and diagnosis of myocarditis in children".)

In children with anomalous origin of the left coronary artery from the pulmonary artery, the typical ECG pattern is of an anterolateral infarction with deep and wide Q waves and T wave inversions in leads I, aVL, V5, and V6 (waveform 2).

Patients with pulmonary hypertension typically have signs of right ventricular hypertrophy and right axis deviation. The ECG may show right ventricular strain (abnormal T waves in the anterior leads).

ECG findings in pulmonary embolism usually are nonspecific ST-T segment changes or sinus tachycardia. If acute right ventricular hypertension occurs, the classic pattern of S wave in lead 1, Q wave in lead III, and T wave inversion in lead III can be seen [11].

Chest radiograph — A chest radiograph should be obtained in children in whom a cardiac or pulmonary disorder or esophageal foreign body is suspected including those with the following findings [3,6] (see 'Physical examination' above and "Causes of nontraumatic chest pain in children and adolescents", section on 'Gastrointestinal'):

Respiratory distress

Pleuritic chest pain

Pathologic cardiac murmur or heart sounds

Pulmonary rales

Hypoxia

Persistent tachypnea (table 3)

History of choking or witnessed ingestion of a foreign body

Signs of cardiac enlargement may be apparent in conditions causing left ventricular outflow obstruction, heart failure, myocarditis, pericarditis, or pericardial effusion.

Patients with pulmonary hypertension may have prominent main and central pulmonary arteries. The peripheral lung fields may be dark if pulmonary vascular resistance is chronically elevated.

In patients with suspected pulmonary disorders, a chest radiograph may show infiltrates caused by pneumonia or areas of atelectasis and air trapping caused by aspiration of a foreign body. Hyperinflation typically is seen in patients with asthma. In addition, pneumothorax, pneumomediastinum, or pleural effusions can be detected.

An anteroposterior and lateral chest radiograph can localize and help identify the type of radio-opaque esophageal foreign body (eg, button battery, coin, or magnet). (See "Foreign bodies of the esophagus and gastrointestinal tract in children", section on 'Imaging'.)

Echocardiogram — Based upon observational studies of pediatric patients referred for cardiology evaluation, an echocardiogram is indicated in the patients with the following findings [17,18]:

History of exertional chest pain or exertional syncope.

Chest pain associated with fever (>38.5°C).

Chest pain that radiates to the back, jaw, left arm or left shoulder, or that increases with supine position.

Past medical history of congenital heart disease, heart transplant, Kawasaki syndrome, or diseases that raise cardiac risk (eg, malignancy, collagen vascular disease, hypercoagulable state, immobilization).

Family history of cardiomyopathy, sudden death, or hypercoagulable state in first-degree relatives (eg, parents or siblings) younger than 50 years of age.

A new murmur, gallop, distant heart sounds, pericardial friction rub, increased intensity of the pulmonary component of S2, or peripheral edema on cardiac examination. (See 'Physical examination' above.)

An abnormal ECG (eg, right or left ventricular hypertrophy, ST segment change >2 mm, low QRS voltage, PR segment depression, S wave in lead 1, Q wave in lead III, or an inverted T wave in lead III).

The utility of echocardiography for such patients includes the following:

Establish the diagnosis of pulmonary hypertension and evaluate ventricular function and associated structural abnormalities.

Determine the severity and site of left ventricular outflow obstruction.

Determine left ventricular function and assess for the presence of dilated or hypertrophic cardiomyopathy.

Assess the size of a pericardial effusion and signs of tamponade, which include variation in Doppler peak velocity across the valves during the cardiac cycle, atrial free wall collapse, or ventricular septal paradoxical motion into the left ventricle during inspiration.

Identify coronary artery abnormalities, including abnormal origin or course, fistula, or aneurysm or stenosis caused by Kawasaki disease.

Diagnose aortic root dissection (image 1). Alternatively, this diagnosis can be made with magnetic resonance imaging (image 2), computed tomography, or aortography (image 3 and image 4A-B). The most efficient imaging technique available at the institution should be used to establish the diagnosis. (See "Clinical features and diagnosis of acute aortic dissection", section on 'Cardiovascular imaging'.)

Diagnose ruptured sinus of Valsalva aneurysm.

Other tests — Less commonly, additional testing may be indicated based upon associated signs and symptoms and the results of the initial evaluation as follows:

Cardiac troponin – Cardiac troponin testing may assist with diagnosis when myocardial infarction or ischemia is suspected. Troponin is a highly sensitive biomarker that aids in the detection of myocardial cell damage, which is often but not always, due to thrombotic obstruction of a coronary artery. Thus, while troponin may be useful to "rule out" a non-ST elevation myocardial infarction, the clinician must recognize the limitations of using troponin to "rule in" MI in patients with low clinical likelihood of an acute coronary syndrome (ACS) which includes most children and adolescents. While troponin is highly specific for myocardial injury, it is not specific for ACS as the cause. As a result, if troponin testing is applied indiscriminately in broad populations with a low pretest probability of thrombotic disease, the positive predictive value for ACS is greatly diminished. Causes of troponin elevations other than acute coronary syndrome are noted in the table (table 5) and discussed in detail separately. In children, myocarditis is frequently the cause of an elevated troponin test. (See "Elevated cardiac troponin concentration in the absence of an acute coronary syndrome".)

Ambulatory cardiac rhythm monitoring – Cardiac rhythm monitoring may be appropriate in some patients with chest pain associated with palpitations, dizziness, or syncope. The type of device for monitoring depends upon several factors and is discussed separately. (See "Ambulatory ECG monitoring".)

However, these tests should be obtained and interpreted in conjunction with a pediatric cardiologist.

APPROACH AND INITIAL MANAGEMENT — The approach and initial management of children and adolescents with chest pain is determined by their clinical status on presentation and findings on history and physical examination (algorithm 2).

Unstable — Although rare in children and adolescents with a primary complaint of chest pain, patients with marked respiratory distress, hemodynamic instability, or sudden collapse require rapid supportive care of the airway, breathing, and circulation according to the principles of Pediatric Advanced Life Support (PALS) (see "Pediatric advanced life support (PALS)") including timely management of the following conditions when present:

Airway foreign body with obstruction – Emergent securing of the airway and treatment of the underlying cause (algorithm 3 and algorithm 4). (See "Emergency evaluation of acute upper airway obstruction in children", section on 'Determining the cause of upper airway obstruction'.)

Tension pneumothorax – Needle or chest tube thoracostomy followed by management of the underlying cause (eg, pulmonary blebs). (See "Spontaneous pneumothorax in children", section on 'Evacuation of the pleural space'.)

Severe status asthmaticus – Rapid administration of oxygen, inhaled bronchodilators (eg, albuterol), and systemic corticosteroid therapy (algorithm 5). (See "Acute severe asthma exacerbations in children younger than 12 years: Intensive care unit management", section on 'Preintubation therapies' and "Acute severe asthma exacerbations in children and adolescents: Endotracheal intubation and mechanical ventilation", section on 'Endotracheal intubation and mechanical ventilation'.)

Pulmonary embolism - Anticoagulant therapy or, in children with a large embolus or hemodynamic compromise, thrombolytics (eg, tissue plasminogen activator or urokinase), or in patients who fail thrombolytic therapy, embolectomy. (See "Treatment, prognosis, and follow-up of acute pulmonary embolism in adults", section on 'Embolectomy' and "Venous thrombosis and thromboembolism (VTE) in children: Treatment, prevention, and outcome", section on 'Pulmonary embolism'.)

Ischemia or infarction – Patients with persistent chest pain consistent with myocardial infarction should receive rapid treatment including anticoagulation, pain management, nitroglycerin, beta blocker if not contraindicated, and, depending upon degree of risk, catheterization (algorithm 6). (See "Initial evaluation and management of suspected acute coronary syndrome (myocardial infarction, unstable angina) in the emergency department", section on 'Management'.)

Tachyarrhythmia – Tachyarrhythmias should be managed according to Pediatric Advanced Life Support principles (algorithm 1). (See "Pediatric advanced life support (PALS)", section on 'Tachycardia algorithm'.)

Heart failure with cardiogenic shock – Patients with heart failure (eg, dilated cardiomyopathy or myocarditis) warrant therapy as determined by the severity of shock (algorithm 7) and degree of failure (table 6). (See "Heart failure in children: Management", section on 'Approach to HF management'.)

Acute chest pain with concerning findings — Most children with a primary complaint of nontraumatic chest pain will be stable at initial evaluation. Those with concerning features of a potential cardiac etiology on history or physical examination (table 1) warrant an ECG (see 'Electrocardiogram' above and "Causes of nontraumatic chest pain in children and adolescents", section on 'Cardiac disease'). Patients with findings of a serious cardiac etiology on physical examination or ECG should have consultation with a pediatric cardiologist and treatment of the underlying abnormality. Patients with a normal physical examination and ECG but a concerning history deserve follow-up with a pediatric cardiologist within one to two weeks.

A chest radiograph is helpful for patients with respiratory distress, pleuritic chest pain, abnormal murmur or heart sounds, or a suspected foreign body. (See 'Chest radiograph' above.)

Pleuritic or positional chest pain suggests pneumonia, pulmonary embolism, spontaneous pneumothorax, pericarditis, and, in patients with sickle cell disease, acute chest syndrome (algorithm 2). These patients warrant timely intervention based upon the underlying cause as follows:

Pneumonia – Supplemental oxygen, endotracheal intubation, and/or mechanical ventilation as needed; hemodynamic support as needed; and antibiotics tailored to the type of pneumonia (eg, community-acquired, nosocomial, or aspiration pneumonia). (See "Pneumonia in children: Inpatient treatment", section on 'Empiric therapy'.)

Pulmonary embolism – Diagnostic testing and imaging to establish the diagnosis and extent of embolism followed by anticoagulant therapy or, in children with a large embolus or hemodynamic compromise, thrombolytics (eg, tissue plasminogen activator or urokinase) or, in patients who fail thrombolytic therapy, embolectomy. (See "Treatment, prognosis, and follow-up of acute pulmonary embolism in adults", section on 'Embolectomy' and "Venous thrombosis and thromboembolism (VTE) in children: Treatment, prevention, and outcome", section on 'Pulmonary embolism' and "Venous thrombosis and thromboembolism (VTE) in children: Risk factors, clinical manifestations, and diagnosis", section on 'Diagnosis'.)

Spontaneous pneumothorax – For patients with large pneumothoraces, significant pain, dyspnea, or hypoxia, needle or chest tube thoracostomy followed by management of the underlying cause (eg, pulmonary blebs). (See "Spontaneous pneumothorax in children", section on 'Evacuation of the pleural space'.)

Pericarditis – Urgent echocardiogram to determine the presence and amount of pericardial fluid, pericardiocentesis in patients with tamponade, and treatment of the underlying cause. (See "Acute pericarditis: Treatment and prognosis", section on 'Adjunctive therapies' and "Acute pericarditis: Treatment and prognosis", section on 'Treatment'.)

Acute chest syndrome – Respiratory support to maintain oxygenation, pain control, bronchodilators for wheezing, broad spectrum antibiotics, and blood transfusion, either simple or exchange transfusion depending upon the degree of severity. (See "Acute chest syndrome (ACS) in sickle cell disease (adults and children)", section on 'Management'.)

Other key findings include history or physical appearance suggestive of Marfan, Ehlers Danlos, Loeys-Dietz, or Turner syndrome indicating a predisposition to aortic root dissection; history of a foreign body or drooling on examination suggesting an esophageal foreign body; pain with swallowing, severe retrosternal pain, and hematemesis seen in patients with esophageal rupture; and fatigue, pallor, weight loss, or abdominal mass in association with a chest wall or intrathoracic neoplasm.

Initial management for these patients consists of the following:

Aortic root dissection – Symptomatic patients warrant emergent evaluation by a pediatric cardiologist and a pediatric cardiothoracic surgeon and typically require emergent surgical intervention. Acute management of these patients is discussed in greater detail separately. (See "Overview of acute aortic dissection and other acute aortic syndromes", section on 'Acute medical management' and "Management of acute type B aortic dissection", section on 'Anti-impulse therapy' and "Management of acute type A aortic dissection", section on 'Acute management'.)

Esophageal foreign body – The management of an esophageal foreign body depends upon the type of object. Symptomatic patients and those with sharp or long foreign bodies, objects in the esophagus for a prolonged period of time, impacted disc batteries, or ingested magnets warrant urgent removal (algorithm 8). (See "Foreign bodies of the esophagus and gastrointestinal tract in children", section on 'Indications for urgent removal'.)

Esophageal rupture – Management of esophageal rupture requires urgent involvement of a surgeon with pediatric expertise. Specific treatment depends upon whether the perforation is free or contained (algorithm 9). (See "Boerhaave syndrome: Effort rupture of the esophagus", section on 'Management'.)

Tumor – Patients with constitutional symptoms such as intermittent fevers, pallor, and weight loss or chest mass on radiography require a work-up designed to assess for the presence of an underlying neoplasm (eg, lymphoma, leukemia, neuroblastoma, or sarcoma). This evaluation should be performed in consultation with a pediatric oncologist. (See "Approach to the adult patient with a mediastinal mass", section on 'Presumptive clinical diagnosis' and "Overview of common presenting signs and symptoms of childhood cancer".)

Common findings — In most pediatric patients, nontraumatic chest pain is not life-threatening. Reassurance is an important component of initial management in these patients. Although the etiology usually is benign, this symptom causes considerable anxiety in patients and their families or caregivers because of the more serious implications of chest pain in adults. Cases with a psychiatric etiology typically need additional counseling and in selected patients, referral to a child psychiatrist.

Important findings of common causes and initial management by etiology include (table 2):

Chest wall pain – Pain on chest wall palpation strongly suggests a musculoskeletal cause such as costochondritis, slipping rib syndrome, or muscle strain from recent exertion or chronic cough. Mild musculoskeletal pain not associated with cough typically responds to analgesics (eg, acetaminophen or ibuprofen) and rest within a few days, although frequently no medication is necessary [6,8].

Patients with chronic cough may need further diagnostic evaluation (algorithm 10). Chest pain usually resolves with treatment of the underlying cause (eg, asthma, cystic fibrosis, postnasal drip, or infection). (See "Approach to chronic cough in children".)

History of stressful event with or without hyperventilation – A history of a recent stressful event (eg, parental divorce or death in the family), sleep disturbance, other somatic complaints (eg, headache or abdominal pain) or physical findings of hyperventilation suggest an underlying psychiatric cause of chest pain. Appropriate initial interventions consist of acknowledgement of the stressful event, reassurance, and follow-up counseling. Patients with known mental illness or significant emotional distress despite reassurance should be referred to a psychiatrist [6]. (See "Causes of nontraumatic chest pain in children and adolescents", section on 'Psychiatric'.)

Fever and cough – Fever, cough, tachypnea, and abnormal lung examination describe findings consistent with viral or community-acquired bacterial pneumonia in stable patients. These patients warrant treatment based upon age and type of pneumonia. Patients with probable bacterial pneumonia warrant empiric oral antibiotic therapy (table 7). (See "Community-acquired pneumonia in children: Outpatient treatment" and "Community-acquired pneumonia in children: Outpatient treatment", section on 'Indications for hospitalization'.)

Cough or dyspnea at night or with exercise – Night-time cough or cough and difficulty breathing with exercise suggests exercise-induced or subclinical bronchospasm. Patients with exercise symptoms that overlap with possible cardiac etiologies warrant an ECG and a chest radiograph. In patients with normal findings, appropriate management consists of a trial of bronchodilators (eg, albuterol) with assured follow-up in patients with known asthma or referral for formal exercise testing in patients without an asthma history. (See "Exercise-induced bronchoconstriction", section on 'Diagnosis' and "Exercise-induced bronchoconstriction", section on 'Management'.)

Heart burn – Substernal burning pain after meals supports the diagnosis of gastroesophageal reflux disease. Patients with frequent and severe pain should be referred to a pediatric gastroenterologist for evaluation of possible esophagitis. Patients with mild symptoms can begin “step up” therapy as described in detail separately. (See "Management of gastroesophageal reflux disease in children and adolescents", section on 'Heartburn'.)

Pain after taking medications – Pain temporally associated with taking medications known to cause esophagitis (eg, tetracycline, doxycycline, aspirin, or nonsteroidal antiinflammatory medications), often without water and at night, strongly suggests pill esophagitis. Whenever possible, the medication should be discontinued. Acid suppression with antacids or histamine-2 receptor blockers (eg, famotidine) should be performed to address any associated gastroesophageal reflux disease. Some patients can have severe pain with swallowing and may not be able to eat or drink. Such patients warrant intravenous hydration and urgent consultation with a pediatric gastroenterologist. (See "Pill esophagitis", section on 'Clinical manifestations' and "Pill esophagitis", section on 'Management'.)

Breast pain – Breast tenderness on palpation in association with gynecomastia in males and thelarche, pregnancy, mastitis, or fibrocystic disease in females. Reassurance is appropriate for patients with gynecomastia and thelarche. (See "Gynecomastia in children and adolescents", section on 'Management'.)

Normal history and physical examination – Patients with idiopathic chest pain have no obvious cause on history and physical examination. They should receive reassurance, anticipatory guidance that the pain may recur, and follow-up with their primary care provider in four to six weeks. (See "Causes of nontraumatic chest pain in children and adolescents", section on 'Idiopathic' and 'Disposition' below.)

INDICATIONS FOR PEDIATRIC SUBSPECIALTY CONSULTATION OR REFERRAL — Children with chest pain and known heart disease or any of the findings that suggest a cardiac cause on history, physical examination, or ECG (table 1) warrant prompt consultation with a pediatric cardiologist.

Urgent consultation with other pediatric specialists is indicated according to the identified condition as follows:

Children with recurrent spontaneous pneumothorax or persistent large air leaks warrant pediatric surgical consultation. (See "Spontaneous pneumothorax in children", section on 'Subsequent management'.)

In addition to the involvement of a pediatric cardiologist, children with aortic root dissection require emergent involvement of a pediatric cardiothoracic surgeon.

Children with pulmonary embolism should undergo evaluation by pediatric specialists with expertise in providing antithrombotic therapy to children and treating the underlying cause of venous thrombosis and thromboembolism (eg, systemic lupus erythematosus, inherited thrombophilia, heart valve disease, or structural venous anomalies).

Patients with sickle cell disease complicated by acute chest syndrome warrant consultation and management by a pediatric hematologist.

Patients with pulmonary hypertension require multidisciplinary consultation with a pediatric cardiologist and pulmonologist.

Other consultations or referrals may be appropriate depending upon the degree of illness and the specific diagnosis. As an example, children with moderate to severe gastrointestinal symptoms, especially those with dysphagia, weight loss, or hematemesis and children with persistent or recurrent pain with no apparent etiology, warrant referral to a gastroenterologist. Evaluation of the upper gastrointestinal tract may reveal esophagitis, gastritis, and/or motility disorders such as diffuse esophageal spasm or achalasia. These abnormalities may be detected in some children with significant chest pain but no gastrointestinal symptoms [19]. (See "Causes of nontraumatic chest pain in children and adolescents", section on 'Gastrointestinal' and "Management of gastroesophageal reflux disease in children and adolescents".)

Children and adolescents with serious emotional disturbance should receive psychiatric consultation.

DISPOSITION — Disposition and follow-up are determined based upon the etiology of the chest pain as follows:

Unstable patients – Initially unstable patients warrant hospitalization in facilities with pediatric intensive care, pediatric cardiology, and other pediatric subspecialty capability. (See 'Unstable' above.)

Stable patients with concerning findings – Patients with abnormal findings suggesting a life-threatening condition should have the disposition determined in consultation with the appropriate specialist. In many of these patients, hospitalization for further testing is appropriate. (See 'Acute chest pain with concerning findings' above.)

Stable patients with common findings – Up to 99 percent of children and adolescents with nontraumatic chest pain have a benign cause, most frequently musculoskeletal chest pain, and can be discharged with additional primary care or specialty follow-up as determined by the underlying etiology. (See "Causes of nontraumatic chest pain in children and adolescents", section on 'Etiology' and 'Common findings' above.)

Idiopathic chest pain may persist or recur. However, serious underlying illness is rare [17,20,21]. As an example, in an observational study that reported on the outcomes of 1938 patients with idiopathic chest pain followed by pediatric cardiologists for a median of four years, none died or had a serious underlying cause identified [17]. Thus, the clinician should avoid extensive ancillary testing in patients with a normal history and physical examination. However, these patients should have periodic follow-up until the pain resolves.

SUMMARY AND RECOMMENDATIONS

Epidemiology – Chest pain is a common symptom in children and adolescents. Its underlying cause is typically benign in this patient population (table 2). (See "Causes of nontraumatic chest pain in children and adolescents", section on 'Epidemiology'.)

Evaluation – A thorough history and physical examination usually can determine the cause and differentiate patients who require further diagnostic studies and acute intervention from those who can be managed with reassurance and continued follow-up. (See 'History' above and 'Physical examination' above.)

Most children with chest pain have no history suggestive of life-threatening conditions (table 1) and either a normal physical examination or findings consistent with a musculoskeletal etiology. Further investigations are not needed in patients for whom a clear etiology, other than cardiac disease, can be established. Diagnostic studies (eg, ECG, chest radiograph, or echocardiogram) are warranted in patients with abnormal physical findings or with associated symptoms (eg, exertional syncope, palpitations, dyspnea, fever, or chest pain associated with exertion) that suggest underlying pulmonary or cardiac disease (table 1). (See 'Ancillary studies' above.)

An ECG should also be obtained when a noncardiac etiology (eg, costochondritis, pneumonia, gastroesophageal reflux, pill esophagitis, or esophageal foreign body) cannot be established. (See 'Electrocardiogram' above.)

Approach and initial management – The approach and initial management of children and adolescents with chest pain is determined by their clinical status on presentation and findings on history and physical examination (algorithm 2). (See 'Approach and initial management' above.)

Children with chest pain and known heart disease or any of the findings that suggest a cardiac cause on history, physical examination, or ECG (table 1) warrant prompt consultation with a pediatric cardiologist. Urgent consultation with other pediatric specialists is indicated according to the identified condition. (See 'Indications for pediatric subspecialty consultation or referral' above.)

Disposition – Up to 99 percent of children and adolescents with nontraumatic chest pain have a benign cause, most frequently musculoskeletal chest pain, and can be discharged with additional primary care or specialty follow-up determined by the underlying etiology. (See 'Disposition' above.)

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Topic 6469 Version 32.0

References

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