Cardiomyopathies Part Two, Dilated
Dilated cardiomyopathies are somewhat common in children and account for .73 cases per 100,000 in the general population. It is a typically inherited disorder, and is often associated with neuro-muscular disorders such as Duchene and muscular dystrophy
Myocarditis is an important aspect of this disease, and is usually viral, but may also be bacterial, fungal, protozoal or parasitic. In other words, this disease may be inherited or acquired.
This disease presents as a dilated, balloon looking or globular heart with atrial enlargement. In the beginning, most patients present with high pulmonary pressures and low cardiac output. Infants usually present poor feeding, and failure to thrive. As the disease progresses, the child will experience dyspnea, tachycardia, peripheral edema and CHF.
The ECG is often normal, but the X-ray is frequently abnormal, reflecting and enlarged heart.
Echocardiography
Evaluate the size of the structures of the heart, the ejection fraction, diastolic function, the presence of thrombus, regurgitation of the valves and pulmonary pressures. Always check for any VSD or ASD, or any outflow obstructions.
Management of this disease is typically prophylactic in order to prevent thrombosis, arrhythmias, and efforts to increase systolic function, for instance beta blockers and digitalis. In the the most extreme cases, ventricular assist devices may be necessary. End stage disease may require may require transplantation.
Ken Heiden RDCS