Restrictive Cardiomyopathy

GENERAL INFORMATION:

What is restrictive cardiomyopathy? Restrictive cardiomyopathy is a disease of the heart muscle that causes the walls of your heart to get stiff. This restricts (holds back) some of the blood and oxygen that is pumped out of your heart to your body.

How does the heart work normally? The heart has 4 chambers (rooms). The two upper chambers are called atria (A-tree-uh) and the two 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 the oxygen into the aorta and out to the body.

How does your heart work with restrictive cardiomyopathy? With restrictive cardiomyopathy, the muscle wall of the left ventricle in your heart gets stiff and is not very flexible. It is difficult for blood to get inside the ventricle because the muscle is so stiff. This means less blood gets pumped out to your body.

Causes: Sometimes it is not known what causes restrictive cardiomyopathy. This disease often happens when something gets inside the muscle tissue, such as protein, iron, or glycogen (sugar). Fibrosis (scarring) of the heart muscle or lining of the heart can cause restrictive cardiomyopathy. Fibrosis can happen after open heart surgery or with illnesses like Loffler's (LEF-lerz) disease. Cancer in the heart muscle can cause restrictive cardiomyopathy. Having radiation treatments to your chest may also cause this disease. The following are diseases that may lead to restrictive cardiomyopathy:

• Amyloidosis (am-eh-loy-DO-sis): Amyloidosis is when fibers (strands) of abnormal protein gather in the heart muscle causing restrictive cardiomyopathy.
  
  
• Sarcoidosis (sar-coy-DO-sis): Sarcoidosis is when inflammation (redness, swelling) causes lumps of tissue to grow in your heart muscle causing restrictive cardiomyopathy.
  
  
• Hemochromotosis (he-mo-kro-mah-TOH-sis): Hemochromotosis is when there is too much iron in your body. With restrictive cardiomyopathy, too much of the extra iron collects in your heart muscle.
  
  
• Scleroderma (skle-ro-DER-mah): Scleroderma is when the heart muscles and small blood vessels get thick, causing restrictive cardiomyopathy.

Signs and Symptoms:

• Abnormal or distant heart sounds.
  
  
• Cough.
  
  
• Crackles heard when caregivers listen to your chest.
  
  
• Fatigue (tiredness).
  
  
• Nausea, bloating, and poor appetite because fluid collects around the stomach, liver, and intestines.
  
  
• Shortness of breath, especially:
  
  
  • With activity.
    
    
  • At night.
    
    
  • When lying flat.
  
  
• Swollen legs and feet caused by retaining fluid.
  
  
• Veins in your neck are distended (stick out).
  
  
• Weakness.

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 repeated as caregivers continue to treat your disease over time. Ask your caregiver for information if you want to know more about the following tests:

• Cardiac catheterization: This is a test to see how well your heart is working. Your arteries (blood vessels) may also be checked to see if they are blocked. A special tube is threaded into your heart through a blood vessel in your leg or arm. Dye may be given so x-ray pictures of your arteries show up better on a TV-like screen.
  
  
• Chest x-ray: This is a picture of your lungs and heart. Caregivers use it to see how your lungs and heart are doing. Caregivers may use the x-ray to look for signs of infection like pneumonia, or to look for collapsed lungs. Chest x-rays may show tumors, broken ribs, or fluid around the heart and lungs.
  
  
• CT Scan: This test is also called a "CAT" scan. A special x-ray machine uses a computer to take pictures of your heart. Your caregivers look at the pictures to see if the ventricle walls have gotten thick. Before taking the pictures, you may be given dye through an IV. The dye helps the inside and outside of your heart show up better in the pictures. People who are allergic (uh-LER-jik) to shellfish (lobster, crab, or shrimp) may be allergic to this dye. Tell your caregiver if you are allergic to any of these.
  
  
• Echocardiogram:
  
  
  • This test is also called an echo. It is a type of ultrasound, using sound waves to show pictures of the size and shape of your heart. An echo also looks at how your heart moves when it is beating. These pictures are seen on a TV-like screen.
    
    
  • This test is done while lying down on your back. Clear jelly will be squirted on your chest to help the ultrasound sensor slide easily. The sensor will be rubbed across your chest to see your heart from different angles. You may hear a barking or whooshing noise, which is the sound of your blood flow. Caregivers may ask you to pedal a bike during the test (exercise echo) or you may get medicine before the test to increase blood flow to your heart muscle (stress echo). This test can tell how well your heart is pumping. An echo can also find problems, such as fluid around the heart or problems with your heart valves.
  
  
• 12-lead ECG: This test, also called an EKG, helps caregivers look for damage or problems in different areas of the heart. Caregivers may need to prepare your skin by shaving off some hair, or cleaning it with a gritty lotion. Sticky pads are placed on your chest, arms, and legs. Each sticky pad has a wire that is hooked to a machine or TV-type screen. A short period of electrical activity in your heart muscle is recorded. Caregivers will look closely for certain problems or changes in how your heart is working. This test takes about 5 to 10 minutes. It is important that you lie as still as possible during the test. You may need this test more than once.
  
  
• Heart 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 the skin over your chest, and put numbing medicine into your skin to make you lose feeling. A small piece of tissue is removed from your heart and sent to the lab for tests. After the biopsy you may need stitches to close the wound. A bandage is then put on the biopsy area.
  
  
• MRI: This test is also called magnetic resonance (REH-zon-nuns) imaging. MRI allows your caregivers to see inside your body. During the MRI pictures are taken of your heart. Caregivers use these pictures to look for thickened ventricle walls.

Treatment Options: Treatment of restrictive cardiomyopathy depends on how much the disease has affected your health. The goal of treatment is to control the problems caused by restrictive cardiomyopathy and improve how you feel from day to day.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 the 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 the problems caused by restrictive cardiomyopathy:
  
  
  • Diuretics (di-u-REH-tiks) to get rid of extra fluid in your body and on your lungs.
    
    
  • Heart medicine to treat a heartbeat that is not normal and to help your heart beat more strongly.
  
  
• Surgery: You may need a heart transplant if you are in good physical condition other than having heart disease. For some people, surgery may help improve blood flow into the heart. You may need a heart transplant if your heart is functioning poorly. Whether you can have a heart transplant depends on what is causing your restrictive cardiomyopathy.

Support:

• Restrictive cardiomyopathy 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 National Center
    7272 Greenville Avenue
    Dallas, TX 75231-4596
    Phone: 1-800-242-8721
    Web Address: http://www.americanheart.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. To help with this plan, you must learn about your health condition and how it may be treated. You can then discuss treatment options with your caregivers. Work with them to decide what care may be used to treat you. You always have the right to refuse treatment.

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