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Congenital Heart Defects - Cardiomyopathy
Cardiomyopathies are diseases of the heart muscle, also known as the myocardium, in which the actual muscle cells and surrounding tissues are sick.
Cardiomyopathies can be primary, meaning the sickness predominantly involves the heart. They can also be secondary, meaning the sickness is a result of another disease or toxin and may also affect many organ systems within the body, including the heart. Patients with cardiomyopathy will most commonly have a heart with normal anatomy.
Cardiologists classify cardiomyopathy into three categories:
1. Dilated cardiomyopathy (DCM): It is also known as congestive cardiomyopathy. Dilated cardiomyopathy is most notable for an enlarged heart that contracts poorly.
2. Hypertrophic cardiomyopathy (HCM): Hypertrophic cardiomyopathy is most notable for thickening of the heart muscle. Some physicians will refer to this class of cardiomyopathy as idiopathic hypertrophic subaortic stenosis (IHSS).
Hypertrophy, or thickening, particularly of the left ventricle, can result in problems with obstruction to forward flow and problems with relaxation of the left ventricle, and can thereby affect the ability of the heart to fill. Children with hypertrophic cardiomyopathy are also at increased risk for arrhythmias.
3. Restrictive cardiomyopathy (RCM): Restrictive cardiomyopathy is most notable for abnormal relaxation of the ventricles and well preserved pumping function. As a result, the upper chambers of the heart, the atria, become enlarged while the lower pumping chambers, the ventricles, remain normal in size. This is a rare form of cardiomyopathy in children.
