Hypertrophic Cardiomyopathy

What is hypertrophic cardiomyopathy?

Hypertrophic (hi-per-TRO-fik) cardiomyopathy (kar-d-o-mi-AH-puh-thee) (HCM) a disease of the heart. In HCM, the heart muscle becomes too thick and too stiff. Sometimes, this thickening only happens in some parts of the heart. When it blocks the flow of blood in the heart it is called idiopathic (ih-d-o-PATH-ik) hypertrophic subaortic (sub-a OR-tik) stenosis (IHSS). Idiopathic means that no one knows what causes the disease.

How does the heart work normally?

The heart has 4 chambers (rooms). The 2 upper chambers are called atria (A-tree-uh) and the 2 lower chambers are called ventricles (VEN-trik-ulls). When the heart "beats", the atria pump blood into the ventricles. The ventricles then pump blood out of the heart. The right ventricle pumps blood into the pulmonary artery, then into the lungs to get oxygen. The left ventricle pumps the blood with oxygen into the aorta and out to your body.

How does your heart work with HCM?

With HCM, the wall of the left ventricle in your heart gets very thick. This makes it difficult for the ventricle to contract (pump) and then to relax. There is not enough space inside the chamber because the muscle is so thick. With so little space, not very much blood can flow into the left ventricle. In some cases, the mitral valve (door) between the left atrium and ventricle gets pushed aside by the large muscle. This makes the mitral valve not open and close correctly. The mitral valve doors and the thick ventricle wall may get in the way of blood being pumped out of your heart.

Causes:

Sometimes it is not known what causes HCM. You may get HCM from your parents' genes. One of your parents may have the gene that causes HCM. Genes are how you inherit or pass down to your children family traits such as eye color and body type. One or more people in your family may have the gene that causes HCM.

Signs and Symptoms:

You may not have any symptoms for awhile after learning that you have HCM. The following signs and symptoms may develop over time as your heart gets larger and weaker.

• Chest pain.
  
  
• Dizziness or fainting with physical activity.
  
  
• Fatigue (tiredness).
  
  
• Strong rapid heartbeats that feel like pounding in your chest.
  
  
• Shortness of breath.

With time, your heart may go into severe heart failure. You may then have one or more of the following signs and symptoms:

• Crackling sound when caregivers listen to your lungs because of extra fluid in your lungs.
  
  
• Swollen legs, feet, and abdomen, and weight gain because of extra fluid in your body.
  
  
• Veins in your neck stick out.
  
  
• Your liver and spleen get larger.
  
  
• Your heart may suddenly start to beat fast and not regularly.

Tests:

Many tests will be done to learn if your heart disease is getting worse. You may have one or more of the following tests. These tests may be done more than once over time. Ask your caregiver for CareNotes™ handout for more information about the following tests:

• Chest x-rays: These x-rays can show if the left atrium and ventricle are larger than the right atrium and ventricle. A chest x-ray can also show fluid on the lungs.
  
  
• EKG: This test is done to learn if your heartbeat is normal. An EKG shows how signals travel through your heart making it contract (pump). This test may be done to see if the signals take too long to travel through your heart.
  
  
• Echo: This test is also called an echocardiogram (eh-ko-KAR-d-o-gram). Sound waves are used to show pictures of the size and shape of your heart. An echo can show caregivers if the left ventricle and left atrium have gotten larger. It also looks at how your heart moves when it is beating and how well the left ventricle is pumping. An echo can also see if the valve (door) between the left atrium and ventricle is opening and closing OK. These pictures are seen on a TV-like screen.
  
  
• Radionuclide (ray-d-o-NU-klid) ventriculography (ven-trik-u-LAW-gruh-fee): This test uses dye to show the size of your left ventricle. It can also show how much blood is pumped out of your heart with each contraction (heartbeat).
  
  
• Cardiac catheterization (kath-uh-ter-ih-ZA-shun): This test measures the pressure inside different parts of the heart.
  
  
• Myocardial (mi-o KAR-d-ull) biopsy (BI-op-c): Caregivers may want to do a biopsy of your heart muscle to help rule out (decide against) other heart problems. Caregivers will clean and put numbing medicine into the skin of your chest to make you lose feeling. A small piece of tissue will be removed from your heart and sent to the lab for tests. After the biopsy, you may need stitches to close the wound. A bandage will be put over the biopsy area.
  
  
• Ultrasound with Color Flow Doppler: This is a test using sound waves to look at your heart. Pictures show up on a TV-like screen. Doppler adds color and sound to show blood flow. You may need the ultrasound to see how large the left ventricle is and how it contracts. The ultrasound also can show if anything is obstructing (stopping) blood flow.

Treatment:

Treatment of HCM depends on how much the disease has affected your health. The goal of treatment is to stop the problems caused by HCM and keep the disease from getting worse. You may have one or more of the following treatments:

• Exercise and diet changes: You may have serious problems with how your heart beats and how blood flows into and out of your heart. These problems may force you to be less physically active. Caregivers may also tell you to eat a diet low in sodium (salt).
  
  
• Medicine: You may need one or more of the following medicines to treat or prevent HCM or the problems caused by HCM:
  
  
  • Antibiotics (an-ti-bi-AH-tiks) to treat or prevent infections (in-FECK-shuns), such as infections of the heart valves. You may have to take antibiotics before having dental care or other types of procedures.
    
    
  • Blood thinning medicine or aspirin to treat or prevent blood clots.
    
    
  • Diuretics (di-u-REH-tiks) to get rid of extra fluid in your body and in your lungs.
    
    
  • Heart medicine to treat an irregular heartbeat and to help your heart beat more strongly.
  
  
• Pacemaker: This is a machine that helps your heart to beat at a normal speed and in a regular rhythm. A pacemaker may be a little box that connects to wires in your heart. A temporary pacemaker can be large patches placed on your chest and back that are connected to a machine. A permanent pacemaker is put under the skin of your chest. You may need a temporary pacemaker only for a short time. You may need a permanent pacemaker for the rest of your life.
  
  
• Surgery: You may need one of the following surgeries if medicines and other treatments do not help.
  
  
  • Myectomy (mi-EK-tuh-me) to cut out part of the enlarged septum (wall) in the left ventricle.
    
    
  • Mitral valve repair or replacement.
  
  
• Cardiac catheterization: You may have treatment through a cardiac cath. Doctors may inject alcohol into part of the heart muscle to make it shrink.

Support:

• HCM is a life-changing disease for you and your family. Accepting that you have heart disease is hard. You and those close to you may feel angry, sad, or frightened. These feelings are normal. Talk to your caregivers, family, or friends about your feelings. Let them help you. Encourage those close to you to talk to your caregiver about how things are at home. Your caregiver can help your family better understand how to support a person with heart disease.
  
  
• You may also want to join a support group. This is a group of people who also have heart disease. Ask your caregiver for the names and numbers of support groups in your town. You can contact one of the following national organizations for more information.
  • American Heart Association
    7272 Greenville Avenue
    Dallas , TX 75231-4596
    Phone: 1- 800 - 242-8721
    Web Address: http://www.heart.org
    
  • National Heart, Lung and Blood Institute
    Health Information Center
    P.O. Box 30105
    Bethesda , MD 20824-0105
    Phone: 1- 301 - 592-8573
    Web Address: http://www.nhlbi.nih.gov/health/infoctr/index.htm
    

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment.

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The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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