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Cardiac emergencies in children are relatively common with congenital heart defects affecting approximately 1% of live births, not accounting for bicuspid aortic valve, which affects approximately 1% of children. In addition, children with structurally normal hearts may have electrophysiologic disturbances that manifest throughout childhood into adulthood, and may acquire heart diseases such as rheumatic fever and myocarditis. Because early in infancy the initial presentation of cardiac disease may be nonspecific, a wide differential should be entertained, including a concern for neonatal sepsis. A family history of sudden cardiac death and congenital heart disease should be obtained. Careful attention to the history and physical examination is essential for the clinician to recognize patterns of illness that may suggest a cardiac etiology for the patient’s presentation.

Clinical Findings

In infants, the most serious congenital heart defects generally present during the first days to 2 weeks of life with cyanosis, congestive heart failure, audible murmurs, and feeding difficulties. In infants, pulmonary volume overload often manifests as wheezing on the lung examination. In children, rales are uncommonly heard acutely and if heard on auscultation represent a very late finding. The absence of an audible murmur most definitely does not rule out the presence of significant cardiac pathology. Later presentations of cardiac disease may manifest in children as palpitations, tachycardia, diaphoresis, tachypnea, hyperpnea, syncope, and, in older children, chest pain.

History and physical examination as well as age of the child may suggest cardiac disease to the clinician’s differential diagnosis. Congenital heart defects may present during stereotyped times during infancy as outlined in Table 35–1. In general, more severe cases of each abnormality will present earlier than milder forms of each disease.

Table 35–1.Congenital heart defects presenting during infancy.

Chest x-ray and electrocardiogram (ECG) are the most important basic studies in evaluating heart disease in children. Although the large cardiothymic silhouette in infants may confuse the diagnostic picture, cardiomegaly on chest radiographs in children should always raise the suspicion for underlying cardiac disease. Pulmonary vascular congestion and volume overload may be very difficult to discriminate from findings of bronchiolitis or other primary pulmonary infection in young children. ECG findings of chamber enlargement or axis deviation should be noted, and rhythm disturbances should be acknowledged and treated as the clinical situation demands.

Laboratory studies to consider include complete blood count (CBC) and metabolic panels, and blood, urine, ...

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