Restrictive Cardiomyopathy

What is restrictive cardiomyopathy?

• Restrictive cardiomyopathy (RCM) is a disease of your heart muscle. Your heart has four chambers (rooms) including two upper atria, and two lower ventricles. The chamber walls are muscle tissue that beat (tighten and relax) constantly. Your atria pump blood into your ventricles. Your right ventricle pumps blood to your lungs to get oxygen. Your left ventricle pumps the oxygen-filled blood to your tissues and organs.
  
  
• With RCM, one or both ventricle walls become stiff. This makes it hard for the ventricles to fill with enough blood. When this occurs, your heart may be unable to pump enough blood and oxygen to your body. You may feel very tired and have shortness of breath when doing your normal activities. When your heart is not able to pump properly, you are at risk for life-threatening heart failure. Treating your RCM may improve your ability to do activities without tiring so easily. Treatment may help make your heart beat stronger and decrease your symptoms. Treatment may prevent your RCM from getting worse and decrease your risk for worsening heart problems.

What causes restrictive cardiomyopathy?

RCM is often caused by another disease but the cause may also be unknown. Diseases can occur in your heart muscle only, or in another body organ that also leads to heart damage. The cause of your RCM may be a genetic disease that was passed on to you from your parents. Your risk for RCM may be increased if you have cancer, diabetes, or if you have had radiation treatments in the past. Ask your caregiver for more information about these and other diseases that may lead to RCM:

• Infiltrative and storage diseases: Infiltrative and storage diseases occur when abnormal substances collect in your heart. These substances include proteins, lumps of cells, and fat. The substances can cause your ventricles to thicken and stiffen, which leads to RCM. These diseases include amyloidosis, Gaucher disease, Fabry disease, and sarcoidosis. Hemochromatosis is also a storage disease that can lead to RCM. This occurs when you have too much iron in your body that collects in your tissues, including your heart.
  
  
• Endomyocardial diseases: With endomyocardial disease, fibrosis (scarring) from radiation treatments, scleroderma, and other conditions occur in your heart. Scleroderma causes hardening of your skin and other tissues. Fibrosis causes the walls of your heart to thicken. Thrombus (blood clots) may also form in your heart causing further damage.
  
  
• Pseudoxanthoma elasticum: Pseudoxanthoma elasticum causes the elastic (stretchy) tissues in your heart to harden.

What are the signs and symptoms of restrictive cardiomyopathy?

You may have any of the following:

• Breathing problems during activity, when you are resting, or both. You may have trouble breathing when you lie down or sleep. You may feel like you cannot get enough air in when you breathe.
  
  
• Chest pain or palpitations (strong, fast heartbeats). You may also feel like your heart is fluttering or jumping in your chest.
  
  
• Distended (bulging) neck veins.
  
  
• Edema (fluid build up). Extra fluid in your body causes weight gain and swelling in your legs, ankles, feet, and abdomen (stomach).
  
  
• Fatigue (tiredness) and feeling very weak.
  
  
• Feeling dizzy or faint when you stand up quickly or when you are exercising.
  
  
• Nausea (upset stomach) and loss of appetite for food.
  
  
• Tenderness (pain when touched) or discomfort in the right side of your abdomen, just under your ribs.

How is restrictive cardiomyopathy diagnosed?

Your caregiver will ask you about your symptoms and any health problems you have. Tell your caregiver if you have family members with RCM or other heart disease. Tell your caregiver if you smoke, drink alcohol, or take any illegal drugs. Your caregiver will check your weight and listen to your heart and lungs. Your caregiver will also check for swelling in your abdomen, ankles, and feet. Your caregiver may check your neck veins (blood vessels) for swelling. You may need different tests to diagnose your RCM, and the cause of your RCM. Ask your caregiver about these and other tests you may need:

• Blood tests: You may need to have your blood taken for tests. Blood tests may help caregivers learn the cause of your RCM. The blood can be taken from a blood vessel in your hand, arm, or the bend in your elbow. You may need to have blood drawn more than once.
  
  
• Cardiac catheterization: This is a procedure done to find the cause of and treat a heart condition. A thin, bendable tube inserted into an arm, neck, or groin vein is moved into your heart. Your caregiver may use an x-ray to guide the tube to the right place. Dye (contrast) may be put into your vein so the pictures show up better on a monitor.
  
  
• Heart monitor: This test is also called an EKG or ECG. Sticky pads are placed on your skin to record your heart's electrical activity. An EKG gives information about how your heart is working. Lie as still as possible during the test.
  
  
• Imaging tests:
  
  
  • Cardiac magnetic resonance imaging: During cardiac magnetic resonance (CMR) imaging, pictures are taken of your heart. Caregivers check your heart, the size of your heart, and the thickness of your ventricles. A CMR can also show if you have iron build-up in your heart. You will need to lie still during this test. Never enter the CMR room with any metal objects. This can cause serious injury.
    
    
  • Chest x-ray: A chest x-ray is a picture of your heart and lungs. Caregivers use the x-ray to check the size of your heart. A chest x-ray is also done to look for fluid around your heart and lungs.
    
    
  • Computed tomography scan: During a computed tomography (CT) scan, an x-ray machine uses a computer to take pictures of your heart. Caregivers check your heart and the size of your ventricles. A CT scan may also show if you have fluid around your heart and lungs. Before taking the pictures, you may be given dye through an IV. People who are allergic to iodine or shellfish (lobster, crab, or shrimp) may be allergic to this dye. Tell your caregiver if you are allergic to shellfish or if you have other allergies.
    
    
  • Echocardiogram: This test is a type of ultrasound. Sound waves are used to show the structure, movement, and blood vessels of your heart.
  
  
• Tissue biopsies: During a biopsy, a small sample of tissue is taken from your body and sent to a lab for tests. A biopsy may be done of your heart or other muscle and tissues in your body. The biopsy can show if you have scarring in your heart and help caregivers learn the cause of your RCM.
  
  
• Urine tests: A sample of your urine may be collected to help learn the cause of your RCM.

What medicines may be used to treat restrictive cardiomyopathy?

• Medicines: You may need any of the following:
  
  
  • Diuretics: Diuretics help your body get rid of edema in your lungs and around your heart. This medicine will also help get rid of extra fluid in your legs and ankles. You may urinate more often when taking diuretics.
    
    
  • Blood thinners: Blood thinners help stop clots from forming in the blood, which may occur with abnormal heartbeats. Clots can cause a stroke, heart attack, and death. Blood thinners may make it easier to bleed or bruise. When taking blood thinners, watch for bleeding from your gums or nose. Watch for blood in your urine and bowel movements. Use a soft toothbrush to prevent bleeding gums. If you shave, use an electric shaver.
    
    
  • Heart medicine: This medicine is given to strengthen or regulate your heartbeat. It also may help your heart in other ways. Talk with your caregiver to find out what your heart medicine is and why you are taking it.
    
    
  • Blood pressure medicine: This is given to lower your blood pressure. A controlled blood pressure helps protect your organs, such as your heart, lungs, brain, and kidneys. Take your blood pressure medicine exactly as directed.
    
    
  • Enzyme replacement therapy: You may be given enzymes (body proteins) to replace lost or low levels of enzymes in your body. This may be done to help treat a condition that caused your RCM.
    
    
  • Iron chelation: Iron chelation is medicine used to decrease iron build up in the body that may have caused your RCM.
    
    
  • Steroids: Steroid medicine may be given to help control problems with your immune system that caused your RCM.
  
  

What treatments may be needed for restrictive cardiomyopathy?

• Surgery: Ask your caregiver for more information about these and other surgeries that may help treat your RCM:
  
  
  • Pacemaker: A pacemaker is an implanted (under your skin) device that helps control abnormal heartbeats.
    
    
  • Implantable cardiac defibrillator: A cardiac defibrillator is implanted under your skin to help prevent life-threatening arrhythmias (abnormal heartbeats).
    
    
  • Heart transplant: During a heart transplant, your diseased heart is removed and replaced with a donor heart. Donors are people who have chosen to give their organs to others after they die.
    
    
  • Left ventricle assist device: A left ventricular assist device (LVAD) is implanted under your skin to help your heart pump better. A LVAD may be needed while you are waiting for a heart transplant.
    
    
  • Heart valve surgery: You may need to have a heart valve repaired or replaced so your heart can pump enough blood to your body. Heart valves act as doors that allow blood flow between your atria and ventricles.
  
  
• Phlebotomy: During phlebotomy, blood is removed, using a vein (blood vessel), to decrease a high iron level in the body. This treatment is done if the cause of your RCM is from iron build-up.

What are the risks of treating restrictive cardiomyopathy?

• Medicines used to treat your heart failure may cause dizziness, low blood pressure, and kidney problems. The levels of salt in your blood may change and lead to abnormal heartbeats. Your heart may beat too quickly or too slowly. If you have surgery to treat your RCM, you may get an infection or bleed more than expected. Even with treatment, your RCM may get worse, and you may die.
  
  
• Without treatment your heart may get weaker and your symptoms may get worse. You may have abnormal heartbeats. You may get a blood clot that travels to your lungs or brain. A blood clot in your lungs can cause chest pain and trouble breathing. A blood clot in your brain can cause a stroke. These problems can be life-threatening. Fluid may build up in your lungs and body. This may make it hard for you to breathe. Your liver and your kidneys may fail. Without treatment, you may die.

How can I help manage my restrictive cardiomyopathy?

• Avoid alcohol: Drinking alcohol can make your heart problem and symptoms worse. Alcohol can cause palpitations and increase your heart rate. Drinking alcohol can also damage your brain, heart, and liver. Alcohol is found in beer, wine, whiskey, and other adult drinks. Talk with your caregiver if you drink alcohol and need help to stop.
  
  
• Exercise and maintain a healthy weight: Exercise may help decrease your symptoms and improve your heart function. Exercise also helps with weight control and keeps your muscles strong. It is best to start slowly and do more as you get stronger. A walking program may help your heart condition. Never start an exercise program without talking with your caregiver. If you are overweight (weighing more than your caregiver suggests), ask your caregiver about an exercise and weight loss plan. Together you can plan the best exercise program for you.
  
  
• Decrease your stress: Having RCM may cause you to have feelings of worry, fear, and sadness. You and those close to you may feel anxious and scared about your future. These feelings are normal. Since it is hard to avoid stress, learn to control it. Learn new ways to relax, such as deep breathing, meditation, and listening to music. Make sure you are also getting enough rest and sleep each day.
  
  
• Check your weight daily: Weight gain can be a sign of extra fluid in your body. Weigh yourself at the same time every morning. It is best to weigh yourself on the same scale, before eating, and after urinating. Record your weights, and the time you weighed yourself in a diary. Bring your diary to your visits with your caregiver.
  
  
• Do not smoke or take illegal drugs: Smoking harms your body in many ways. Smoking can make your heart condition worse and cause other heart disease, lung disease, and cancer. Quitting smoking will improve your health and the health of those around you. Ask your caregiver for more information about how to stop smoking if you are having trouble quitting. Do not take any illegal street drugs, such as cocaine and methamphetamines. Street drugs can make your heart condition and symptoms worse.
  
  
• Limit your liquids and eat a healthy diet: Follow your caregiver’s advice about how much liquid you should drink each day. Drinking too much liquid can increase your risk for fluid build up and swelling. You may need to limit the amount of salt and fat you eat each day. Talk with your caregiver about what amount of salt and fat is okay for you. Together you can plan the best diet for your health.
  
  
• Manage your other medical conditions: Get proper treatment and use all of your medicines to control other health problems as directed by your caregiver. These conditions include diabetes and high blood pressure.

Where can I find more information?

Contact the following:

• 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
  

When should I call my caregiver?

Call your caregiver if:

• You are gaining weight for no known reason.
  
  
• You are weaker or more tired than usual.
  
  
• You have chest pain or trouble breathing that is getting worse over time.
  
  
• You have increased swelling in your legs, ankles, feet, or abdomen.
  
  
• You have questions or concerns about your condition, treatment, or care.

When should I seek immediate help?

Call 911 or seek immediate help if:

• You feel dizzy or you faint (pass out) after standing up or when you are exercising.
  
  
• You feel like your heart is fluttering or jumping in your chest.
  
  
• You have more trouble breathing than usual, or you cannot breathe well when lying down or exercising.
  
  
• Your heart is beating much faster than is normal for you.
  
  
• Your neck veins are bulging (sticking out).
  
  
• You are urinating very little, or not at all.
  
  
• You have new and sudden chest pain. You may have more pain when you take deep breaths or cough. You may cough up blood.
  
  
• Call 911 or an ambulance if you have any signs of a heart attack:
  
  
  • Discomfort in the center of your chest that feels like squeezing, pressure, fullness, or pain, that lasts for more than a few minutes or keeps returning
    
    
  • Discomfort or pain in your back, neck, jaw, stomach, or one or both of your arms
    
    
  • Feeling sick to your stomach
    
    
  • Having trouble breathing
    
    
  • A sudden cold sweat, particularly in combination with chest discomfort or trouble breathing
    
    
  • Feeling very lightheaded or dizzy, particularly in combination with chest discomfort or trouble breathing
  
  
• You have signs of a stroke: The following signs are an emergency. Call 911 immediately if you have any of the following:
  
  
  • Weakness or numbness in your arm, leg, or face (may be on only one side of your body)
    
    
  • Confusion and problems speaking or understanding speech
    
    
  • A very bad headache that may feel like the worst headache of your life
    
    
  • Not being able to see out of one or both of your eyes
    
    
  • Feeling too dizzy to stand

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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