Topic Overview

What is restrictive cardiomyopathy?

Restrictive cardiomyopathy is a serious problem that makes your heart muscle stiff. When your heart muscle is stiff, it can't stretch to allow enough blood to enter its lower chambers, the ventricles . So blood that would normally enter the heart backs up in your circulatory system.

Most of the time, this leads to heart failure . Heart failure doesn't mean that your heart stops pumping. It means that your heart can't pump enough blood to meet your body's needs.

What causes restrictive cardiomyopathy?

Often the cause is never found. But we do know that there are a number of diseases or problems that can lead to restrictive cardiomyopathy. These include:

• Cardiac amyloidosis , a buildup of an abnormal protein in the heart muscle.
• Hemochromatosis , a buildup of iron in the heart muscle.
• Sarcoidosis , a rare type of heart inflammation.
• Radiation therapy and chemotherapy , used to treat cancer.
• Carcinoid syndrome , a rare disease that causes certain chemicals to be released into the bloodstream. These chemicals can stiffen heart muscle.
• Löeffler's syndrome and endomyocardial fibrosis, conditions that can cause scar tissue in the heart.
• Genetic factors. You can inherit diseases, including Gaucher disease and Fabry's disease , that can lead to restrictive cardiomyopathy. But these diseases can be treated to prevent restrictive cardiomyopathy.

What are the symptoms?

You may not have any symptoms at first. Or you may have mild symptoms, such as feeling very tired or weak.

If your heart gets weaker, you will develop heart failure. When this happens, you will feel other symptoms, including:

• Shortness of breath, especially with activity.
• Tiredness.
• Trouble breathing when you lie down.
• Swelling in your legs.

Heart failure that suddenly gets worse is an emergency. Get medical help right away if:

• You have severe trouble breathing.
• You cough up pink, foamy mucus.
• You have a new irregular or rapid heartbeat.

When you have heart failure, keeping track of your symptoms every day is important. Call your doctor if:

• You have a sudden weight gain, such as more than 2 lb (0.9 kg) to 3 lb (1.4 kg) in a day or 5 lb (2.3 kg) in a week. (Your doctor may suggest a different range of weight gain.)
• Your ability to exercise changes.
• You have any change in your symptoms.

How is restrictive cardiomyopathy diagnosed?

Your doctor will ask questions about your symptoms and past health. He or she will want to know about recent illnesses and about heart disease in your family. Your doctor will listen to your heart and lungs and check your legs for fluid buildup.

You may also have other tests, including:

• Electrocardiogram , also known as an ECG or EKG.
• Chest X-ray .
• Echocardiogram .
• Cardiac catheterization .
• Routine blood tests.

In some cases, a doctor may want to look at a small sample of heart tissue, called a biopsy , to make a definite diagnosis.

How is it treated?

Most of the time, treatment focuses on relieving symptoms, improving heart function, and helping you live longer. You may also have other treatment for the problem that is causing restrictive cardiomyopathy, such as medicines to get rid of too much iron in the heart muscle ( hemochromatosis ).

You will probably need to take several medicines to treat heart failure caused by restrictive cardiomyopathy. It's important to take your medicines exactly as your doctor tells you to and to keep taking them. If you don't, your heart failure could get worse.

Your doctor may suggest a mechanical device to help your heart pump blood or prevent life-threatening irregular heart rhythms. Such devices include a pacemaker , an implantable cardioverter-defibrillator (ICD) , or a combination pacemaker and ICD. If your condition is very bad, a heart transplant may be an option.

Self-care is an important part of your treatment. Self-care includes the things you can do every day to feel better, stay healthy, and avoid the hospital.

• Take your medicines as prescribed. This gives you the best chance of being helped by them. Some medicines for heart failure include:
  • Angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers (ARBs). These make it easier for blood to flow.
  • Diuretics. These help remove excess fluid from the body.
  • Beta-blockers. These slow the heart rate and can help the heart fill with blood more completely.
• Live a healthy lifestyle. This can help slow down heart failure. Limit salt, and don't smoke. Ask your doctor about how you can exercise safely. People who have heart failure from restrictive cardiomyopathy need to avoid doing too much, because their hearts can't increase blood flow during exercise.
• Watch for signs that you're getting worse. Weighing yourself every day to watch for sudden weight gain is a good way to do this.
• Find out what your triggers are, and learn to avoid them. Triggers are things that make your heart failure worse, often suddenly. A trigger may be eating too much salt, missing a dose of your medicine, or exercising too hard.

What can you expect with restrictive cardiomyopathy?

Most of the time, restrictive cardiomyopathy leads to heart failure. Heart failure usually gets worse over time. But treatment can slow the disease and help you feel better and live longer. If your doctor finds the cause of your restrictive cardiomyopathy, then the cause will also be treated, if possible.

Some people develop other problems, including:

• Stroke .
• Heart attack .
• A blood clot in the lung, called a pulmonary embolism .
• Sudden cardiac death, which means the heart suddenly stops working. This may be more likely to happen to people who have serious rhythm problems ( arrhythmias ) in one of the lower heart chambers ( ventricles ).

If your disease is getting worse, you may want to think about making end-of-life decisions. It can be comforting to know that you will get the type of care you want.