Sudden Death Syndrome and Congenital HCM

Almost half a million people in the United States have a common congenital heart problem called hypertrophic cardiomyopathy or HCM and it is a leading cause of sudden death, particularly in people with no history of health problems.

This heart disease afflicts the heart muscle itself and the result is that the walls of the lower ventricles of the heart become thickened. When this thickness occurs, it is enough to cause your heart to work abnormally.

In addition, the thicker walls of the ventricles can cause these lower heart chambers to twist or warp, thus impairing the operation of the mitral and aortic valves, the ones that control your blood flow to and from the heart.

HCM can be blamed on heredity and it starts by creating a problem with the development of the fibers in the heart muscle. Usually only one parent needs to carry this genetic trait in order for you to get this disease.

However, at least 50% of the cases of HCM did not inherit this heart condition. Instead a genetic mutation occurs. With this unexpected mutation, you could pass it along to your children, even though your siblings and parents don’t have it.

Likely Problems that HCM Causes

HCM aka hypertrophic cardiomyopathy can cause several different types of heart problems for you. Systolic dysfunction is one problem that stems from HCM. In this case, the heart does not pump the blood properly so there is not enough blood expelled. Deformed ventricles can affect the aortic or mitral valve causing this systolic dysfunction.

Diastolic dysfunction is the result of the HCM thickening and stiffening the ventricles of the heart which makes it much harder for them to fill with blood so that it can be pumped out to the rest of the body. Blood may take a reverse path back into the lungs when any major physical activity or exertion of the body is made.

Heart failure can also result from HCM and is usually due to the heart muscle thickening far beyond what it can cope with and still function. This condition is called dilated cardiomyopathy and it occurs late in cases of HCM.

Sudden death is another problem and is usually the result of major tachycardia or what you may term as heart flutters. Sudden death will many times occur in the midst or after major physical exertion.

Diagnosis and Treatment

Diagnosis of HCM is best through the application of an echocardiogram or EKG. This test can measure the thickness of the ventricle walls as well as identify anomalous functions of the aortic and mitral heart valves. If you know of any close family members that have HCM, you should immediately schedule an echocardiogram as the disease can be passed down in the family.

There is no cure for HCM but the condition can be treated through the use of different heart medications. Blockers like calcium or beta blockers can help keep the walls of the heart ventricles more pliant. Surgery to remove some of the thickened areas of the ventricle walls is an option as well. Prevention is not an option so diligence in regular check-ups and testing is necessary.