Cardiomyopathy

Cardiomyopathy literally means disease of the heart muscle or the myocardium. In this disease, the heart loses its ability to pump blood and beat at a normal rhythm. The condition tends to start off mild and then worsens fairly quickly. In the most severe cases, cardiomyopathy can lead to congestive heart failure. The main types of Cardiomyopathy are: Dilated or ‘enlarged’ heart, the most common form of Cardiomyopathy occurs when the heart muscles become weak and cannot pump blood effectively.

The weak muscles relax, and allow the chambers of the heart to expand. Most cases of dilated cardiomyopathy are a result of coronary artery disease, but about 30 percent of cases are genetic in origin. Dilated Cardiomyopathy occurs in 2 out of 100 people. It occurs most often in middle age, and it affects more men than women. However, this disease can occur in people of all ages, including children. Although dilated cardiomyopathy often occurs in people with no family history of the disease, about 30 percent of cases are thought to have genetic causes.

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Hypertrophic or ‘thickened muscle’; In hypertrophic cardiomyopathy, the heart muscle cells become enlarged. In fact, the word “hypertrophy” literally means enlargement. This enlargement results in a thickening of the walls of the heart, which then prevents the heart from functioning properly. In hypertrophic cardiomyopathy, the heart muscle cells become enlarged. In fact, the word “hypertrophy” literally means enlargement. This enlargement results in a thickening of the walls of the heart, which then prevents the heart from functioning properly.

Arrhythmogenic right ventricular, a more unusual form associated with arrhythmias; is a very rare cardiomyopathy that occurs in one out of every 5,000 people. In this disease, the muscle of the right ventricle is gradually replaced by a layer of fatty tissue. This fatty tissue causes major problems with the heart’s rhythm. The most common result of this disease is cardiac sudden death, in which the heart suddenly stops beating. In fact, it accounts for one fifth of all cases of cardiac sudden death in people younger than 35, and is the leading cause of sudden death in young athletes.

Although not a lot is known about the disease, somewhere between 30 to 90 percent of cases are inherited in a dominant fashion. This means that a person only has to inherit a mutated version of the gene from one parent in order to be at risk. Restrictive or ‘stiff’ heart, the least common occurs when the heart walls become stiff and can’t relax enough to fill with blood. A heart that cannot fill with blood cannot effectively pump blood to the body. The most common cause of restrictive cardiomyopathy is when a protein called amyloid builds up in the heart muscle.

However, it also occurs as a result of hemochromatosis or as a result of other heart diseases, some of which are inherited. This type of cardiomyopathy is relatively rare, occurring in 1 out of 1000 people. Treatment In most cases, doctors cannot treat cardiomyopathy once it develops. For this reason, it is important to be screened regularly if you have a family history of the disease or if you have other risk factors. Some patients with advanced dilated cardiomyopathy may require a heart transplant. Of those who do get a heart transplant, 75 percent live an additional five years.

In the United States donor hearts are scarce, so those who qualify for heart transplantation often have to wait months, or even years, for a suitable match. While waiting, some people with declining heart function can use a small, implanted mechanical pump called a left ventricular assist device (LVAD) to take over much of the heart’s blood pumping activity. Unfortunately, LVADs only work temporarily, so they are not a good substitute for transplant Genetic testing At this time there is no genetic test for the one gene that is known to cause dilated cardiomyopathy.

If you have a family history of the disease, your doctor will probably recommend that you adopt lifestyle changes that are recommended for the general population, such as exercising, not smoking, and not drinking excessively. Your doctor may also recommend that you be screened for heart disease more regularly than people in the general population. http://www. cmaa. org. au/whatis. html http://www. genetichealth. com/HD_What_is_Dilated_Cardiomyopathy. shtml http://www. genetichealth. com/hd_what_is_cardiomyopathy. shtml

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