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Page 1 of 2 Hypertrophic Cardiomyopathy
Hypertrophic Cardiomyopathy (HCM) is a genetically transmitted disease that directly affects the heart muscle. Because of several high–profile cases of young athletes dying suddenly of HCM, it is more commonly known as a disease of athletes.
However, this disease can affect anyone – men, women, and children of all ages – regardless of their physical condition. HCM is the second most common form of cardiomyopathy, occurring in roughly 0.2% of the general population. The risk of sudden death from HCM is 2–3% per year. HCM is characterized by an abnormal thickening of the walls of the heart. Some thickening is normal for healthy persons who exercise or who play competitive sports. In HCM, however, the thickening becomes extreme. In some patients with HCM, the septum, which separates the left and right sides of the heart, bulges into the left ventricle. Muscles in both ventricles often become enlarged, and these thickened muscle walls may partially block the flow of blood through the aortic valve or prevent the heart from stretching properly and filling with blood. In a small percentage of HCM patients, the mitral valve separating the left atrial and ventricular chambers may also be damaged or may stick to the septum, which causes an obstruction to blood flow through the valve.
Causes
A hereditary link has been established for this form of cardiomyopathy. In about half the cases, the person has inherited an abnormal gene from a parent, in the rest of the cases, a gene that is normal at birth later mutates. Fortunately, a genetic test can identify who may be at risk for HCM. Using the results of this test, doctors can warn children at risk to avoid extra exertion and refrain from participating in certain competitive sports. Other causes for HCM have been traced to hypertension and heart valve disease.
Symptoms
Fainting during strenuous exercise is often the first and most dramatic symptom of this disease. Other symptoms (if they occur) are shortness of breath, chest pain, and fatigue. Persons who experience these warning signs should seek immediate medical attention. Young athletic adults who die of HCM usually die before they are diagnosed with the condition. HCM also may be more difficult to diagnose in athletes because it is similar to a benign condition called left ventricular hypertrophy, an enlargement of the left ventricle that often develops in association with strenuous physical conditioning. Anyone – young or old – who wants to participate in strenuous athletic competition should have a thorough physical exam that includes an Electrocardiogram (ECG or EKG) and chest X–ray.
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