Hypertrophic Cardiomyopathy

What do doctors call this condition?

Idiopathic hypertrophic subaortic stenosis

What is this condition?

This primary disease of the heart is marked by disproportionate, asymmetrical thickening of the interventricular septum, the muscular wall that separates the heart's chambers. It may go undetected for years but may lead to potentially fatal heart failure.

What causes it?

This disorder results from defective cells in the heart muscle, a problem that may be genetic in origin. Signs and symptoms occur because the thickened heart muscle impedes the flow of blood in and out of the heart.

What are its symptoms?

Generally, symptoms don't appear until the disease is well advanced. The person may experience chest pains, irregular heart rhythms, shortness of breath, fainting, heart failure, and sudden death.

How is it diagnosed?

Diagnosis of hypertrophic cardiomyopathy depends on typical clinical findings and on test results:

. Echocardiography (most useful) shows increased thickness of the interventricular septum and abnormal motion of the heart valves .

. Electrocardiography usually reveals changes in the electrical impulses generated by the heart and altered heart rhythms.

How is it treated?

The goals of treatment are to relax the ventricle and to relieve obstructed blood flow in and out of the heart. The drug Inderal helps to slow the heart rate and relax the obstructing muscle, thereby promoting adequate filling of the heart's ventricle. This helps to reduce angina, fainting episodes, shortness of breath, and irregular heart rhythms.

If drug therapy fails, surgery is indicated. Ventricular myotomy (removal of the enlarged septum) alone or combined with replacement of the mitral valve may ease obstructed blood flow and relieve symptoms. However, ventricular myotomy is a risky procedure.