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Hypertrophic Cardiomyopathy

Alternate Names

  • hypertrophic obstructive cardiomyopathy
  • idiopathic hypertrophic subaortic stenosis
  • Normal Heart
  • Cardiomyopathy

Definition

Hypertrophic cardiomyopathy is a condition in which a portion of the heart muscle is abnormally thick. This can make it harder for blood to flow into and out of the heart, resulting in a variety of symptoms.

What is going on in the body?

One of the main blood-pumping chambers of the heart, called the left ventricle, is affected in hypertrophic cardiomyopathy. For unknown reasons, the walls of this heart chamber become abnormally thick. The heart chamber becomes stiff, which makes it harder for blood to flow in and out of the heart.

Risks

What are the causes and risks of the condition?

The cause is of hypertrophic cardiomyopathy is unknown, but most cases are thought to be inherited. Someone who has a close relative with this condition has a much higher risk than others.

Prevention

What can be done to prevent the condition?

There are no known ways to prevent hypertrophic cardiomyopathy.

Diagnosed

How is the condition diagnosed?

After a complete history and physical exam, the healthcare provider may order tests to help confirm the diagnosis of hypertrophic cardiomyopathy. These tests may include:
  • an electrocardiogram (ECG)
  • a chest X-ray
  • an echocardiogram, an imaging test that uses ultrasound waves to see the beating heart. This test confirms the diagnosis by showing the abnormally thick walls of the heart.

Long Term Effects

What are the long-term effects of the condition?

The long-term effects of hypertrophic cardiomyopathy are hard to predict. There is an increased risk of abnormal heartbeats, called arrhythmias, and heart infections, called endocarditis.
Hypertrophic cardiomyopathy also causes an increased risk of sudden death. This risk is not related to how severe the person's symptoms are. Most cases or sudden death are thought to be due to arrhythmias, but why they occur remains unexplained.

Other Risks

What are the risks to others?

Hypertrophic cardiomyopathy is not contagious, but it may be inherited by an affected person's children. Genetic counseling may be helpful to couples with a family history of this condition.

Treatments

What are the treatments for the condition?

The goals of therapy are to reduce symptoms of hypertrophic cardiomyopathy and prevent death. Because sudden death has been linked to exercise, strenuous exercise should be avoided, whether or not the person has symptoms.
Heart medications are often used to reduce symptoms such as chest pain, palpitations, and fainting. Commonly used medications include atenolol (i.e., Tenormin), amiodarone (i.e., Cordarone, Pacerone)), and diltiazem (i.e., Cardizem, Cartia, Dilacor, Tiazac).
A type of pacemaker device called a defibrillator may also be used for dangerous abnormal heartbeats. Minor surgery is needed to put a defibrillator in place.
For someone with severe symptoms that cannot be controlled by medication, injection of alcohol through a catheter into the thickened part of the heart is sometimes effective. Or, open heart surgery may be advised. The surgery involves removing part of one of the thickened walls of the heart.

Side Effects

What are the side effects of the treatments?

All medications have possible side effects. For example, atenolol may cause sleepiness and erectile dysfunction. Diltiazem may cause stomach upset or swelling in the legs. Surgery can be complicated by infection, bleeding, and allergic reaction to anesthetic.

After Treatment

What happens after treatment for the condition?

The exact course of hypertrophic cardiomyopathy is hard to predict. Treatment is usually needed for long periods of time, sometimes for life.

Monitor

How is the condition monitored?

Any new or worsening symptoms should be reported to the healthcare provider. Regular visits to the healthcare provider allow ongoing monitoring for arrhythmias or worsening of the hypertrophic cardiomyopathy. Repeat electrocardiograms (ECGs) and echocardiograms are often used to help with monitoring.

Sources

Merck Manual, 1999

Current Medical Diagnosis and Treatment, 1996

Harrison's Principals of Internal Medicine, 1991

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