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Nature of HOCM/IHSS

HOCM (hypertrophic cardiomyopathy, idiopathic hypertrophic subaortic stenosis) is a complex genetically determined disease of the heart muscle that is present in as many as 1 in 500 people in the general population. It is characterized by thickening of the heart muscle with a normal sized heart cavity. It is diagnosed by an echocardiogram. In most people, the disease has a good outlook with few symptoms and a normal life expectancy. However, in some patterns of the disease there is an increased risk of premature death, heart failure and rhythm disturbance.

Evaluation

A comprehensive team approach is emphasized. The patients are seen in our center by cardiologists with extensive experience of these problems. Echocardiography is performed by highly specialized staff using the latest 3-D echo technology. Heart catheterization is usually performed. Rythym problems are assessed by cardiac electrophysiologists. The team is based at the Methodist-DeBakey Heart Center at the world renowned Methodist Hospital in Houston Texas

Treatment Options

In mild cases, after careful evaluation, no treatment may be needed. In many other cases, medications such as beta blockers and calcium channel blockers can be used effectively. In more severe cases, the excessive muscle buildup in the outflow tract of the left ventricle may need to be removed. This can be done in some cases by injecting alcohol into one of the coronary arteries running into the lump of muscle and destroying that area. If this is not possible, unsuccessful or if other problems are present, such as blocked heart arteries or a valve problem, heart surgery is often recommended. In some cases where the risk of sudden death is present, automatic implantable cardiovertes defibrillators (AICD) are recommended. The surgery is also combined with coronary bypass surgery where indicated. The low risk nature and benefits of HOCM surgery are presented in the editorial of Dr. Maron, a world authority on HOCM, and Dr. Woo.


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