Staten Island Heart's Doctors, Board Certified Cardiologists, Specializing in the treatment, care and prevention of Cardiovascular Disease. Located in Staten Island, New York.
Hypertrophic Cardiomyopathy
What is hypertrophic cardiomyopathy?
Hypertrophic cardiomyopathy (HCM) is a disease that causes enlargement and thickening of the heart muscle. The thickening may be so severe that it interferes with the proper pumping of blood from the heart. There are several names for HCM including idiopathic hypertrophic subaortic stenosis (IHSS) and asymmetric septal hypertrophy (ASH). In this disease, there is a thickening of individual heart muscle cells. The cells may become tangled and "jumbled up" instead of having their normal pattern. These changes can occur throughout the heart or only in a portion of it. When the muscle gets too thick, it decreases the size of the left ventricular pumping chamber and increases the stiffness of the heart. This impairs the ability of the heart to relax and accept blood into the main pumping chamber, decreasing the heart's ability to pump. A localized thickening of the muscle just below the aortic valve may cause an obstruction to the flow of blood out of the heart and into the aorta.
Who gets HCM?
HCM seems to run in families, and the disease is most likely the result of a gene defect. There may be other factors that also influence the disease, but it is most closely associated with the genes that control heart muscle growth. People of all ages have HCM, but younger people are likely to have a more severe form of the disease.
What are the symptoms of HCM?
HCM varies widely in how it affects people. Many people have no symptoms at all. Others may be nearly incapacitated. The most frequent symptoms are shortness of breath with physical activity and chest pain. Dizziness is common, particularly when standing suddenly, and fainting can occur. Heart rhythm disturbances may occur in this disorder and may be life threatening. HCM is a major cause of death in young athletes who die during heavy exercise.
How is HCM diagnosed?
HCM can be suspected on the basis of history and a physical examination. A heart murmur may be heard. A heart murmur is a sound produced by blood flowing past an obstruction. Some patients with HCM may have a heart murmur all of the time. Other patients may have a murmur occasionally, or not all. Your doctor also will look for special characteristics of your pulse in the arms and neck.
An echocardiogram, or cardiac ultrasound, is a very useful test to make the diagnosis of HCM. This study provides a precise measure of the thickness of the heart muscle and can measure the degree of obstruction present. Cardiac catheterization, a hospital study utilizing small catheters and x-rays, can further characterize the disorder and its response to medications. Routine studies include a chest x-ray and an electrogram, a 24-hour tape recording of your electrocardiogram made by a portable recorder to look for heart rhythm problems resulting from HCM.
Because the disease runs in families, your doctor may suggest that the other members of your family be tested for HCM.
What are the risks of HCM?
Doctors cannot accurately predict the course of the disease in HCM patients. Therefore, the risk of sudden death or congestive heart failure must be considered. When the physical examination, the echocardiogram, and the 24-hour tape recording of the electrocardiogram show no blockage to blood flow or potentially dangerous heart rhythm irregularity, the risk is low. Regular, routine observation by your doctor may be all that is needed. For patients at higher risk of complications or who are symptomatic with the disease, further treatment may be necessary. The disease may result in congestive heart failure and life threatening heart rhythm disturbances.
How is HCM treated?
Treatment depends on the degree of risk. There is no present treatment for the gene defect. Your doctor must treat the effects of the disease. Most congestive heart failure in HCM patients results from obstruction to blood outflow or from abnormal heart muscle relaxation. The usual congestive failure medicines can be used for HCM but may not be totally effective. Drugs that assist the relaxation phase of the heartbeat (calcium channel blockers) or that mildly reduce the abnormal heart contraction (beta-blockers, disopyramide) have been helpful. A recent effective treatment has been the insertion of a permanent pacemaker which can alter how the heart contracts thus improving the obstruction to blood flow. An earlier therapy involved a surgical procedure called myectomy (muscle removal), where a strip of the thickened heart muscle was surgically removed to help relieve the obstruction. This procedure is still occasionally performed. At times one of the heart valves, the mitral valve, is replaced surgically in conjunction with the myectomy.
Identification of HCM patients with heart rhythm problems and potential sudden death is very important. The treatment of rhythm disorders uually requires medications. Occasionally, an implantable defibrillator may be necessary to prevent sudden death.
Who gets HCM?
HCM seems to run in families, and the disease is most likely the result of a gene defect. There may be other factors that also influence the disease, but it is most closely associated with the genes that control heart muscle growth. People of all ages have HCM, but younger people are likely to have a more severe form of the disease.
What are the symptoms of HCM?
HCM varies widely in how it affects people. Many people have no symptoms at all. Others may be nearly incapacitated. The most frequent symptoms are shortness of breath with physical activity and chest pain. Dizziness is common, particularly when standing suddenly, and fainting can occur. Heart rhythm disturbances may occur in this disorder and may be life threatening. HCM is a major cause of death in young athletes who die during heavy exercise.
How is HCM diagnosed?
HCM can be suspected on the basis of history and a physical examination. A heart murmur may be heard. A heart murmur is a sound produced by blood flowing past an obstruction. Some patients with HCM may have a heart murmur all of the time. Other patients may have a murmur occasionally, or not all. Your doctor also will look for special characteristics of your pulse in the arms and neck.
An echocardiogram, or cardiac ultrasound, is a very useful test to make the diagnosis of HCM. This study provides a precise measure of the thickness of the heart muscle and can measure the degree of obstruction present. Cardiac catheterization, a hospital study utilizing small catheters and x-rays, can further characterize the disorder and its response to medications. Routine studies include a chest x-ray and an electrogram, a 24-hour tape recording of your electrocardiogram made by a portable recorder to look for heart rhythm problems resulting from HCM.
Because the disease runs in families, your doctor may suggest that the other members of your family be tested for HCM.
What are the risks of HCM?
Doctors cannot accurately predict the course of the disease in HCM patients. Therefore, the risk of sudden death or congestive heart failure must be considered. When the physical examination, the echocardiogram, and the 24-hour tape recording of the electrocardiogram show no blockage to blood flow or potentially dangerous heart rhythm irregularity, the risk is low. Regular, routine observation by your doctor may be all that is needed. For patients at higher risk of complications or who are symptomatic with the disease, further treatment may be necessary. The disease may result in congestive heart failure and life threatening heart rhythm disturbances.
How is HCM treated?
Treatment depends on the degree of risk. There is no present treatment for the gene defect. Your doctor must treat the effects of the disease. Most congestive heart failure in HCM patients results from obstruction to blood outflow or from abnormal heart muscle relaxation. The usual congestive failure medicines can be used for HCM but may not be totally effective. Drugs that assist the relaxation phase of the heartbeat (calcium channel blockers) or that mildly reduce the abnormal heart contraction (beta-blockers, disopyramide) have been helpful. A recent effective treatment has been the insertion of a permanent pacemaker which can alter how the heart contracts thus improving the obstruction to blood flow. An earlier therapy involved a surgical procedure called myectomy (muscle removal), where a strip of the thickened heart muscle was surgically removed to help relieve the obstruction. This procedure is still occasionally performed. At times one of the heart valves, the mitral valve, is replaced surgically in conjunction with the myectomy.
Identification of HCM patients with heart rhythm problems and potential sudden death is very important. The treatment of rhythm disorders uually requires medications. Occasionally, an implantable defibrillator may be necessary to prevent sudden death.
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