Abstract
Background
Chest pain is a common presenting symptom in pediatric emergency rooms and clinics. The wide spectrum of causes may create a diagnostic conundrum for clinicians.
Aim of review
The purpose of this article is to review cardiovascular causes of pediatric chest pain with illustrative case histories and discussion of pathophysiology, diagnosis, and treatment.
Key scientific concepts of review
Guidelines for cardiovascular screening include the American College of Cardiology and American Heart Association 14-element checklist, American Academy of Pediatrics recommendations with four questions about cardiac symptoms, signs, and family history, and international guidelines for electrocardiogram interpretation. Noncardiac chest pain may be caused by various conditions, such as exercise-induced asthma. Pericarditis may be acute or chronic and caused by various conditions, including viral infection. Sudden cardiac arrest may occur without previous chest pain and most commonly is caused by hypertrophic cardiomyopathy. Anomalous coronary artery is a rare congenital defect that may cause exertional chest pain or sudden cardiac arrest and may require multidisciplinary care for diagnosis and treatment. Myocarditis may be caused by viral infection, may present with tachycardia, chest pain, difficulty breathing, and cardiovascular instability, and may be diagnosed with endomyocardial biopsy or cardiac magnetic resonance imaging. Spontaneous pneumothorax may present with sharp chest pain in tall, thin adolescent boys, and treatment may include close observation with serial chest radiographs, oxygen therapy, simple aspiration, or chest tube placement. Aortic dissection is a rare, life-threatening condition associated with connective tissue disorders and may necessitate percutaneous endovascular grafting or aortic replacement surgery. Pulmonary embolism may occur in pediatric patients, and risk factors include sepsis, current oral contraceptive use, and recent surgery. Prompt diagnosis and treatment of conditions causing chest pain may be facilitated with a comprehensive history, detailed physical examination, and ancillary studies.
Highlights
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Chest pain is a common presenting symptom in pediatric emergency rooms and clinics.
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Pericarditis is the most common cause of pediatric cardiac-related chest pain.
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Hypertrophic cardiomyopathy may cause sudden cardiac arrest in young athletes.
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Coronary artery anomalies are rare but also may cause sudden cardiac arrest.
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Myocarditis, pneumothorax, aortic dissection, and pulmonary embolism are emergencies.
1
Introduction
Chest pain is a frequent presenting symptom in pediatric emergency rooms and office visits, accounting for 1 in 40 clinic visits, but it rarely is caused by cardiac disease [ ]. Most pediatric chest pain is caused by noncardiac etiologies, such as musculoskeletal, idiopathic, respiratory, psychogenic, gastrointestinal, and miscellaneous conditions [ ] ( Table 1 ). A guide to help pediatric primary care providers differentiate noncardiac versus cardiac causes of chest pain in children was published previously [ ]. A careful chart review, history, and physical examination may provide clues to enable diagnosis of noncardiac causes of chest pain [ ]. The wide spectrum of causes may create a conundrum for clinicians in the diagnostic evaluation of chest pain that may be caused by occult cardiovascular disease, including life-threatening conditions.
| Category | Diagnosis |
|---|---|
| Musculoskeletal (most common) | Costochondritis (Tietze syndrome) Muscle strain Precordial catch syndrome Slipping rib syndrome |
| Idiopathic (diagnosis of exclusion) | Unexplained chest pain |
| Respiratory, pulmonary | Acute chest syndrome (sickle cell disease) Asthma Chronic cough Infection
Pleuritis Pneumomediastinum Pneumothorax Pulmonary embolism Pulmonary hypertension |
| Psychogenic | Anxiety disorders Conversion disorder Depression Panic disorder |
| Gastrointestinal | Crohn’s disease Esophagitis Foreign body ingestion Gallbladder disease Gastric ulcer Gastritis Gastroesophageal reflux disease Hiatal hernia Pancreatitis |
| Miscellaneous | Breast disease Mediastinitis Shingles |
The purpose of this article is to review cardiovascular causes of pediatric chest pain. We used a case-based strategy to facilitate an understanding of inherent differences in the history, clinical presentation, and evaluation between various cardiovascular diseases that may cause chest pain and require prompt treatment. Although some electrocardiogram (ECG) findings are mentioned, a complete review of arrhythmias is beyond the scope of this article, and further information about arrhythmias is available in other articles in this issue of the journal [ ].
2
Guidelines for evaluation
In 2014, the American College of Cardiology and American Heart Association released a 14-element cardiovascular screening checklist for congenital and genetic heart disease in youth, which included seven components of personal history, three components of family history, and four components of the physical examination ( Table 2 ) [ ]. In 2021, the updated American Academy of Pediatrics recommendations for screening all youths every three years, during the preparticipation evaluation, or upon entry to middle, junior high, or high school, regardless of athletic status, included four questions about cardiac symptoms, signs, and family history ( Table 2 ) [ ], and patients with an affirmative response are evaluated further with an ECG and pediatric cardiology referral [ ].
| Guideline | Item | |
|---|---|---|
| 14-element screen for congenital and genetic heart disease a | ||
| Personal history | ||
| 1 | Exertional chest pain | |
| 2 | Unexplained syncope | |
| 3 | Exertional dyspnea, fatigue, or palpitations | |
| 4 | Prior heart murmur | |
| 5 | Elevated blood pressure | |
| 6 | Previous restriction from sports participation | |
| 7 | Previous cardiac testing ordered by a physician | |
| Family history | ||
| 8 | Premature death in a relative before age 50 years from cardiac disease | |
| 9 | Disability from cardiac disease in a close relative aged <50 years | |
| 10 | Family member with inheritable cardiovascular disease, hypertrophic or dilated cardiomyopathy, long QT syndrome, other ion channelopathies, Marfan syndrome, clinically important arrhythmia | |
| Physical examination | ||
| 11 | Heart murmur | |
| 12 | Femoral pulses suggesting coarctation of the aorta | |
| 13 | Physical stigmata of Marfan syndrome | |
| 14 | Elevated brachial artery blood pressure while sitting | |
| 4 screening questions about sudden cardiac arrest | ||
| 1 | Fainted, passed out, or had unexplained seizure, especially during exercise or after sudden loud noises? | |
| 2 | Any previous exercise-related chest pain or shortness of breath? | |
| 3 | Any immediate family member died of heart problems or unexpected sudden death before age 50 years (e.g., unexpected drowning, car accident while driving, or sudden infant death syndrome)? | |
| 4 | Any relative with hypertrophic cardiomyopathy, hypertrophic obstructive cardiomyopathy, Marfan syndrome, arrhythmogenic right ventricular cardiomyopathy, long or short QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, or younger than 50 years with pacemaker or implantable defibrillator? | |
a Adapted from American College of Cardiology and American Heart Association [ ].
International guidelines for interpretation of ECGs in athletes include ECG findings that indicate the possible presence of occult cardiovascular disease and are indications for further diagnostic evaluation for conditions that may be associated with sudden cardiac arrest ( Table 3 ) [ ]. ECG is also indicated for evaluation of cardiac causes of chest pain when there is a history of moderate to severe COVID-19 infection within the previous 6 months or multisystem inflammatory syndrome in children [ ]. Furthermore, a normal ECG may not exclude all cardiovascular pathology and may appear normal with anomalous coronary arteries, cardiovascular disease associated with connective tissue disorders, and some cardiomyopathies, arrhythmias, or ion channelopathies. Therefore, recommendations about further evaluation also may depend on the systematic history, physical examination, and clinical judgment [ ].
| Electrocardiogram finding |
|---|
| T-wave inversion |
| ST-segment depression |
| Pathologic Q waves |
| Complete left bundle branch block |
| QRS duration ≥140 ms |
| Epsilon wave |
| Ventricular preexcitation |
| Prolonged QT interval |
| Brugada pattern, type 1 |
| Profound sinus bradycardia <30 beats per minute |
| PR interval ≥ 400 ms |
| Mobitz type II second-degree atrioventricular block |
| ≥ 2 premature ventricular contractions |
| Atrial tachyarrhythmia |
| Ventricular tachyarrhythmia |
≥ 2 of these borderline findings:
|
Although 99 % of pediatric patients who are evaluated for chest pain in the emergency room may have noncardiac causes, it is important to identify the 1 % of patients who have cardiac-related chest pain because this may help prevent sudden cardiac arrest [ , ]. Other studies showed that cardiac causes of chest pain may be identified in 0.6 % to 8 % of children presenting with chest pain [ , ]. Sudden cardiac arrest in young patients is rare, with 0.6 to 6.2 deaths per 100,000 children and adolescents, with the most common causes being related to the development of a final common pathway including ventricular tachycardia or fibrillation caused by hypertrophic cardiomyopathy, coronary artery abnormalities, myocarditis, congenital heart disease, or inherited arrhythmias [ ]. Furthermore, it is important to recognize other cardiovascular causes of chest pain, such as aortic dissection, pulmonary embolism, and pneumothorax promptly for immediate intervention to prevent death [ ].
3
Cases
3.1
Case 1. Teenaged girl with exercise-induced chest pain
3.1.1
History and examination
A 16-year-old girl plays softball and complains of recurrent bilateral chest tightness at 15 to 20 min after starting exercise. She can finish the games but develops shortness of breath. She denies palpitations, dizziness, syncope, or seizure and has a negative family history. As a toddler, she had a history of respiratory syncytial virus and required albuterol treatments but currently is not on any therapy. Physical examination findings are unremarkable, and spirometry is normal. An ECG is obtained ( Fig. 1 ). What is the most likely diagnosis?
3.1.2
Diagnosis and treatment
The patient had noncardiac chest pain caused by exercise-induced asthma. The presenting symptoms of chest tightness and chest pain occurring after initiation of exercise may be caused by exercise-induced bronchospasm or cardiac etiologies. When chest pain occurs during exercise, it is important to distinguish between exercise-induced asthma and cardiac disease because both problems may present with shortness of breath with exercise [ ].
In this case, the history was suggestive of noncardiac chest pain because the patient had bilateral chest tightness and pain, whereas cardiac chest pain typically may be isolated to the left side. Patients who have other forms of noncardiac chest pain, such as costochondritis or chest wall pain, most frequently have tenderness elicited on palpation of the sternum or ribs [ ]. Cardiac chest pain typically is not aggravated by palpation because there are no nervous system connections between the chest wall and heart.
Bronchial asthma is an atopic disease, and environmental irritants in the home, including smoke, pets, stove use, mold, or wool, may increase the risk of developing asthma [ ]. >5 million children in the US have bronchial asthma diagnosed, and this disease causes >750,000 emergency room visits annually [ ]. Exercise-induced asthma is characterized by airway narrowing during or after physical activity, usually triggered by inhaling polluted air, allergens, or cold, dry air that may cause airway cooling, dehydration of airway surfaces, and irritation and inflammation of the airway epithelium [ ].
Diagnosing exercise-induced asthma may be challenging because the patient may have normal lung function at rest. Evaluation includes spirometry with a bronchodilation test. Criteria for the diagnosis of asthma include an increase in the ratio of forced expiratory volume in one second to forced vital capacity (FEV1:FVC) of the predicted value for age [ ]. If the bronchodilation test is negative but there is suspicion of the presence of exercise-induced asthma, other provocative tests such as the methacholine challenge test, exercise test, and hyperosmolar test with mannitol or saline may help establish the diagnosis [ ]. Another etiology in the differential diagnosis is vocal cord dysfunction, which is characterized by abnormal motion of the vocal cords during exercise and associated difficulty breathing with exertion [ ].
Recommendations for patients with exercise-induced asthma include warming up for 10 to 15 min before participating in sports. Pharmacotherapy may include inhaled corticosteroids as monotherapy or in combination with long-acting β-agonists [ ]. Adolescents with mild asthma are advised to use as-needed inhaled corticosteroids and formoterol, maintenance inhaled corticosteroids combined with as-needed short-acting β-agonists, or as-needed inhaled corticosteroids and formoterol as an alternative to as-needed short-acting β-agonists [ ].
3.2
Case 2. Teenaged boy with sharp chest pain
3.2.1
History and examination
A male football player aged 15 years wakes up in the morning with sharp left-sided chest pain and difficulty breathing at rest, especially when lying flat. He was sick one month ago with upper respiratory tract symptoms that resolved, but otherwise, he previously was healthy. He comes to the emergency room, and examination shows that he is afebrile and the heart rate is 100 beats per minute, respiratory rate 25 breaths per minute, blood pressure 120/76 mmHg, and oxygen saturation 98 % as measured by pulse oximetry. Chest radiography shows mild cardiomegaly. ECG shows diffuse PR-segment depression and generalized ST-segment elevation, and the patient has an echocardiogram ( Fig. 2 ). What is the most likely diagnosis?
3.2.2
Diagnosis and treatment
The patient had acute pericarditis. Pericardial disease is the most common cause of cardiac-related chest pain in children (87 %) [ ]. Pericarditis may be classified as acute, with new-onset symptoms persisting less than four to six weeks; incessant, symptoms persisting >6 weeks but <3 months; chronic symptoms persisting >3 months; and recurrent, recurrence after a four- to six-week symptom-free period [ ]. The etiologies of pericarditis in children include viral infection, cardiac surgery, neoplasia, renal disease, and rheumatologic diseases. Autoimmune conditions, including systemic lupus erythematosus and juvenile arthritis, may cause recurrent pericarditis [ ]. Pericarditis also has been reported in patients with COVID-19 and as a rare adverse event of the COVID-19 vaccine [ ].
Pericarditis presents with chest pain in 95 % of affected children, frequently accompanied by fever, fatigue, and dyspnea [ ]. Chest pain typically may be relieved when sitting upright or leaning forward but may be exacerbated while lying supine. Examination may show Kussmaul sign, which is an increase in the jugular venous pressure during inspiration, and a pericardial knock, which is an extra heart sound during rapid filling in diastole. Patients also may have pulsus paradoxus, which is a decrease in the systolic blood pressure during inhalation caused by a decrease in left heart filling and cardiac output associated with pericardial effusion with tamponade or constrictive pericarditis. The Beck triad includes muffled heart sounds, jugular venous distension, and hypotension and may be observed in patients who have pericarditis with cardiac tamponade [ ].
Laboratory studies may show increased serum inflammatory marker levels, including erythrocyte sedimentation rate and C-reactive protein level, and elevated cardiac enzyme levels may occur in 30 % of patients who have pericarditis with concurrent myocarditis [ ]. ECG changes may include PR-segment depression and widespread ST-segment elevation. Echocardiography may show and quantify a pericardial effusion and differentiate constrictive pericarditis from restrictive myocarditis. Cardiac magnetic resonance imaging (MRI) and cardiac computed tomography scans may be considered when the echocardiogram is inconclusive or to exclude the associated presence of myopericarditis. Cardiac computed tomography with contrast may show pericardial inflammation, thickening, and calcification and associated pneumonia. Cardiac MRI is the noninvasive test of choice for detection of localized myocardial edema and myopericardial inflammation [ ]. Diagnosis of pericarditis typically is made when at least two of the four findings are present: chest pain, pericardial friction rub, ECG changes, and pericardial effusion [ ].
First-line treatment of pericarditis in pediatric patients includes nonsteroidal anti-inflammatory drugs, colchicine, and exercise restriction. Commonly used nonsteroidal anti-inflammatory drugs in children include ibuprofen, indomethacin, and naproxen [ ]. Evidence for colchicine treatment in pediatric pericarditis is based mostly on adult studies; although it is used frequently in adults, it is typically considered in children only for treatment of recurrent pericarditis [ ]. Exercise restriction is recommended for 3 months in patients who have uncomplicated pericarditis and 6 months for pericarditis with concurrent myocarditis [ ]. Glucocorticoids and immunotherapies have been used successfully in the treatment of pediatric pericarditis, but glucocorticoids are used with caution because they have been associated with increased rates of recurrent pericarditis and glucocorticoid dependence. Patients who have glucocorticoid dependence or colchicine resistance may be treated with immunotherapies such as azathioprine, intravenous immunoglobulin, rilonacept, and anakinra [ , ].
3.3
Case 3. Teenaged girl with sudden cardiac arrest
3.3.1
History and examination
A female flag football player aged 17 years has sudden cardiac arrest on the field. The emergency medical team treats her with cardiopulmonary resuscitation and an automated external defibrillator, and the resuscitation is successful. After she is brought to the emergency room, the ECG shows left ventricular hypertrophy and T-wave inversion in the left and inferior leads. Family history includes atrial fibrillation and a thickened heart in a paternal relative. Cardiac magnetic resonance imaging is performed. What is the most probable underlying cause?
3.3.2
Diagnosis and treatment
The patient had hypertrophic cardiomyopathy, which is among the main causes of death and the most common cause of sudden cardiac arrest in young athletes [ , ]. It is a genetic disorder affecting the sarcomere proteins, with abnormalities in the contractile unit of the myocyte that may create the phenotype of left ventricular hypertrophy [ ]. A mutation in sarcomere protein genes may be present in 1 of every 300 to 500 people, but phenotypic expression is variable [ ]. Most patients who have hypertrophic cardiomyopathy have mutations affecting two main structural proteins: MYBPC3 , which encodes myosin binding protein-C, and MYH7 , which encodes β-myosin heavy chain [ ].
The hallmark of this disease is the development of progressive left ventricular remodeling, which may cause decreased coronary perfusion of the thickened myocardium, myocardial fibrosis, and risk of developing ventricular arrhythmias [ , ]. The left ventricular hypertrophy most commonly begins at puberty but may begin at any age. A thorough family history is important because the disease may be acquired through autosomal dominant inheritance [ ].
Pediatric patients with hypertrophic cardiomyopathy typically present with a range of symptoms that may vary with age of onset and severity of the condition [ ]. Clinical manifestations in infancy may include progressive myocardial hypertrophy and circulatory failure, resulting in symptoms such as difficulty breathing, poor feeding, and failure to thrive [ ]. Older children may be asymptomatic or develop symptoms such as chest pain, shortness of breath, palpitations, a new heart murmur, or syncope, especially during physical exertion [ ]. Adolescents may exhibit exercise intolerance and arrhythmias, but sudden cardiac arrest may be the first sign [ ]. The heterogeneity in presentation underscores the importance of obtaining a thorough family history in all new patients [ ].
Pediatric patients with hypertrophic cardiomyopathy may have murmurs that may be best heard when the patient is standing or performing a Valsalva maneuver because of associated decreased venous return, and cardiac examination may be helpful in supine, sitting, and standing positions [ ]. Preparticipation sports screening with an ECG may also detect occult cardiomyopathies and decrease the risk of developing sudden cardiac arrest in athletes [ ]. The ECG may detect T-wave abnormalities in >95 % of patients who have hypertrophic cardiomyopathy, often before the development of left ventricular hypertrophy [ ]. Characteristic ECG findings include increased left-sided forces, Q waves in the inferior leads, deeply inverted T waves, T-wave abnormalities in the precordial leads, and voltage criteria for left atrial enlargement [ ] ( Fig. 3 ).
