What is Hypertrophic Cardiomyopathy (HCM)?
To better understand what Hypertrophic Cardiomyopathy, HCM, is lets first talk about how cardiomyopathies are described.
Cardiomyopathy is a condition in which the muscle of the heart is abnormal in the absence of an apparent cause. This terminology is purely descriptive and is based on the Latin deviation. HCM is a primary and usually familial cardiac disorder with heterogeneous expression, unique pathophysiology, and a diverse clinical course, for which several disease causing mutations in the genes encoding proteins of the cardiac sacomere have been reported. There are some forms of HCM, that are currently being debated by the medical community regarding how they should specificly be defined because the causethe hypertrophy in these cases is now understood to be from other mutations impacting either the storage of glycogen or lysosomal storage within the heart.
While HCM has typically been recognized by its structure ie., hypertrophy, the electrical function of the heart are also adversely affected. There are four types of cardiomyopathy: "hypertrophic", "dilated", "restrictive" and "right ventricular".
The main feature of hypertrophic cardiomyopathy is an excessive thickening of the heart muscle (hypertrophy literally means to thicken). Thickening is seen in the ventricular septal measurement (normal range .08-1.2cm), and in weight. In HCM, septal or wall measurements may be in the range of 1.3cm to 6.0+cm anywhere in the left ventricle. Heart muscle may also thicken in normal individuals as a result of high blood pressure or prolonged athletic training. Furthermore, there is a fine line between and athletic heart, hypertensive heart disease and a heart with HCM.
In Hypertrophic Cardiomyopathy (HCM), the muscle thickening occurs without an obvious cause. In addition, microscopic examination of the heart muscle in HCM is abnormal. The normal alignment of muscle cells is absent and this abnormality is called "myocardial disarray".
These diagrams contrast the regular, parallel alignment of muscle cells in a normal heart with the irregular, disorganized alignment of muscle cells or "myocardial disarray" found in some parts of the heart in hypertrophic cardiomyopathy.
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