Services and Programs

Children's Heart Center at Strong

Specialized Programs - Cardiomyopathy Clinic

Cardiomyopathies are disorders of the muscle (myocardium) that performs the pumping action of the heart. In cardiomyopathy, the diseased heart muscle can become thickened, thinned, stiff, unable to normal conduct electrical impulses or just poorly functioning. Over time the weakened heart fails to pump adequately resulting in heart failure or irregular heartbeats (arrhythmias or dysrhythmias).

The causes of cardiomyopathy are different in the pediatric population than in adults and often require different management. In the pediatric age range this group of disorders is a result of multiple causes including infection and inflammation of the heart, inherited disorders of the proteins that contribute to heart muscle function and injuries to heart such as from cancer therapy, malnutrition, inadequate blood flow, structural heart disease, accumulation of materials that cannot be metabolized in heart muscle and iron overload.

While the major therapies used in the management of adult cardiomyopathies, beta-blockers and afterload reduction, may be helpful in many of the patients, there are new therapies being developed specifically for disorders that occur in the pediatric age range. These include idebenone therapy for the hypertrophic cardiomyopathy seen in Friedreich ataxia and enzyme replacement therapy for Pompe’s disease. In the Pediatric Cardiomyopathy Clinic a pediatric cardiologist will provide comprehensive medical management of the patient’s condition for optimal results in close association with other specialists such as electrophysiologists, pediatric endocrinologists and geneticists.

 

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