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This chapter reviews the most common types of acquired heart disease in children: inflammatory and infectious disorders, and cardiomyopathies, of which the most important is hypertrophic cardiomyopathy (HCM).


Myocarditis is an inflammatory disorder of the myocardium affecting children of all ages and is the leading cause of end-stage cardiomyopathy requiring transplantation. Viral causes include enteroviruses (coxsackie, echovirus, and poliovirus), as well as mumps, influenza virus, and varicella zoster virus. Emerging causes are human immunodeficiency virus–associated myocarditis and chronic Epstein-Barr myocarditis. Many bacterial species have been associated with myopericarditis but not myocarditis alone. Noninfectious causes include conditions such as Kawasaki disease (see discussion below in Kawasaki Disease) and lupus erythematosus.

Clinical Features

Myocarditis is often preceded by a viral respiratory illness. Presenting signs and symptoms are often respiratory distress, fever, tachypnea, tachycardia, generalized malaise, fever, and myalgias. Vomiting, decreased activity, and poor feeding are present. Arrhythmias may complicate myocarditis and give rise to symptoms of palpitation or syncope in older children. Chest pain may be a symptom of concurrent pericarditis.1–3

The physical examination reveals signs of decreased cardiac output and compensatory response: tachycardia, weak pulses, cool extremities with delayed capillary refill, skin mottling, or cyanotic skin. Auscultation of the heart may reveal distant heart sounds, an S3 or S4 gallop, and a regurgitant murmur. There may be signs of congestive heart failure.


Diagnostic evaluation includes complete blood count, serum chemistries, and blood cultures to identify bacterial infection. Inflammatory markers, such as erythrocyte sedimentation rate and C-reactive protein, are nonspecific but may be elevated. Respiratory viral cultures or viral titers may help to identify a specific infectious cause. Troponin T or I may be elevated.

ECG changes are nonspecific, with sinus tachycardia, low QRS voltages (<5 mm in limb leads), flattened or inverted T waves with ST- and T-wave changes, and prolongation of the QT interval. Left ventricular hypertrophy or strain can be seen. Arrhythmias include premature ventricular contractions, atrial tachycardias, junctional tachycardia or, occasionally, heart block, or ventricular tachycardia.1–3

Chest x-ray may reveal cardiomegaly and pulmonary edema, and echocardiography is useful to define cardiac function. Biopsy is the diagnostic gold standard.


Treatment of myocarditis depends upon the cause. Treatment of heart failure also depends on the specific cause, and treatment modalities need to be individualized. Treatment consists of afterload reduction, preload reduction in the case of true volume overload, or inotropic support. Diuretics will worsen the condition if cardiac output depends upon preload.1–3 Children with heart failure need admission to a tertiary care pediatric facility.


Pericarditis is inflammation of the pericardium and has many causes. Infectious causes are common and can be bacterial, viral, fungal, parasitic, or tubercular. Viral etiologies predominate in the infant. Pericarditis is often associated with myocarditis, with myocarditis the predominant entity. Staphylococcus aureus, Streptococcus pneumoniae...

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