CHAPTER 105

Chest Pain

Robin Winkler Doroshow, MD, MMS, MEd, FAAP

Chest pain in children is among the most common reasons for referral to a pediatric cardiologist, second only to heart murmurs. It is, however, 1 of the least common presenting symptoms of cardiac disease. In fact, from an etiologic standpoint, it would be more suitable to include this chapter in the section on gastrointestinal disorders, respiratory problems, psychological and psychogenic disorders, or hematology, rather than cardiology (Figure 105.1).

Perhaps the strongest reason for addressing chest pain in the cardiology section, along with discussions of heart murmurs, cyanosis, and congestive heart failure, is that it is the greatest fear of the patient, parent or caregiver, and physician that chest pain could herald a serious—even lethal—heart problem in an otherwise healthy pediatric patient. This fear is exacerbated by 2 social phenomena in the United States: the widespread press given to cases of sudden unexpected death in young, apparently healthy athletes (who had not, in fact, experienced chest pain in most cases), and the appropriately high level of concern for the underdiagnosis of ischemic heart disease in the adult population. Public information targeting the adult with chest pain may, unfortunately, be misinterpreted as applying to the child as well. It is this apprehension, and the associated medicolegal defensive posture, that so often drives the patient and the referring primary care physician to seek cardiology consultation and testing. The frequency of such referrals has increased 4- to 5-fold in the past 2 decades, resulting in increasingly inefficient resource utilization.

Epidemiology

The epidemiology of pediatric chest pain is not well studied. Published retrospective studies have attributed chest pain to cardiac causes in 0.25% of patients treated in emergency departments (EDs), 0.3% treated in an outpatient clinic, and 1% treated in a pediatric cardiology clinic.

In the primary care office setting, chest pain is the third most frequent pain symptom, after abdominal pain and headache, accounting for more than 600,000 office visits a year in the United States. The most common age range is 8 to 18 years, peaking between 11 and 13 years of age. No sex or ethnic predilection exists, although the etiologic distribution may vary by sex.

Cardiac causes of chest pain are quite uncommon for 2 reasons. Such pain is either a rare symptom in a common cardiac disorder (eg, aortic stenosis) or caused by a rare condition (eg, anomalous origin of coronary artery [AOCA]).

Clinical Presentation

Substantial variability exists in the symptom of chest pain in children (Box 105.1). Some pediatric patients experience a single, protracted, severe episode that results in a visit to the ED. In that setting, the patient may have other signs or symptoms of an acute medical problem, such as fever, shortness of breath, or hypotension, raising the level of suspicion for a serious organic cause.

More frequently, children report repeated episodes occurring sporadically over a period of months or even years. The pain may be induced or exacerbated by exercise, deep breathing, lying down, or eating. It can begin and end abruptly or gradually. It may be accompanied by other symptoms, which may help in making a diagnosis. In cases in which the pain occurs with exercise, the physician must determine whether it is a consistent, predictable experience, or is unpredictable, as well as whether it is associated with dizziness or syncope.

Figure 105.1. Causes of chest pain in a series of 3,700 pediatric patients seen for this symptom during a 10-year period.

Reprinted with permission from Saleeb SF, Li WYV, Warren SZ, Lock JE. Effectiveness of screening for life-threatening chest pain in children. Pediatrics. 2011;128(5):e1062–e1068.

Up to 97% of children evaluated for chest pain have no serious medical history, and most have an unremarkable physical examination.

Pathophysiology

The pathophysiology varies based on the underlying cause of the pain, and the differential diagnosis is diverse (Box 105.2). It is important for the patient, parent or caregiver, and physician to understand that thoracic or abdominal pain, and visceral pain in particular, is poorly localized. This makes it difficult to distinguish between esophagitis and cardiac ischemia, for example; hence, the term “heartburn” for the former condition. Precordial pain may originate from any intrathoracic organ or from the chest wall itself, and the patient cannot identify the source of the discomfort, which in itself is a cause of distress.

^a Common cause of chest pain.

^b Uncommon cause of chest pain.

^c Rare cause of chest pain.

Cardiac causes are a heterogeneous category that are best characterized as ischemic or nonischemic in origin (Box 105.3). None of these causes is common. Ischemic chest pain, or angina, is caused by a drop (often sudden) in the myocardial oxygen supply/demand ratio, causing pain or, in rare cases, sudden death. This may occur because of diseased coronary arteries as in adults, resulting from accelerated coronary atherosclerosis, but such an occurrence is quite rare in the pediatric population. Angina may occur in the patient with progeria, mucopolysaccharidosis, post-transplantation coronary disease, or severe familial hypercholesterolemia. Alternatively, it may occur as a result of previous inflammation (as with coronary aneurysm in Kawasaki disease), resulting in coronary artery stenosis. A congenital AOCA from the opposite sinus of Valsalva (Figure 105.2) or from the pulmonary artery may result in ischemia resulting from compression of the anomalous vessel between the great arteries, obstruction from a slit-like orifice at the origin, or, in the case of the pulmonary artery, low pressure and low saturation in the perfusing blood.

The chest pain that occurs in a small minority of patients with severe left ventricular outflow obstruction (ie, aortic valve stenosis and hypertrophic obstructive cardiomyopathy) likely is also the result of ischemia. The already diminished supply/demand ratio resulting from hypertrophy and hypertension of the left ventricle falls further with strenuous exercise, causing pain and/or arrhythmia.

With use of drugs, such as cocaine, chest pain may be attributable to arrhythmia, myocardial hypoperfusion, or a combination of these. This may present as chest pain or overt collapse, in some cases with cardiac arrest.

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