Restrictive Cardiomyopathy

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 does not 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 blood stream. 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.
• Chest pain.

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

• Have severe shortness of breath.
• Have a fast or uneven heartbeat.
• Cough up foamy, pink mucus.
• Have chest pain.

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.
• 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?

You will probably need to take several medicines to treat heart failure caused by restrictive cardiomyopathy. It is very 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.

Lifestyle changes are an important part of your treatment. Taking these steps can help slow down heart failure.

• Limit how much salt you eat. Salt causes water to build up in your body and makes it harder for your heart to pump. Limit your fluid intake if your doctor tells you to.
• Limit your physical activity. Talk to your doctor about the best balance of rest and activity.
• Limit how much alcohol you drink.

Your doctor may suggest a pacemaker or other device to help your heart beat normally. If your condition is very bad, a heart transplant may be an option.

Keeping track of your symptoms every day is an important part of your treatment. Call your doctor if:

• You have a sudden weight gain of 1 kg (2 lb) in a day or 2.5 kg (5.5 lb) in a week.
• Your ability to exercise changes.
• You have any sudden change in your symptoms.

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. In more and more cases, the problem is being found earlier, when it can be better managed.

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 over time, 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.

Frequently Asked Questions

Learning about restrictive cardiomyopathy:	What is restrictive cardiomyopathy? What are the symptoms?
Being diagnosed:	How is restrictive cardiomyopathy diagnosed?
Getting treatment:	How is restrictive cardiomyopathy treated? Are heart transplants an option for people with this disease?
Ongoing concerns:	How will my doctor monitor my heart failure? How do I avoid triggers for sudden heart failure? How often will I see my doctor if I have heart failure?
Living with restrictive cardiomyopathy:	What can I do to take care of myself? How do I cut back on fluids? How do I cut back on sodium?
End-of-life issues:	How can I help make decisions about long-term care? How can I prepare for end-of-life issues?

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Author:	Douglas Dana Robin Parks, MS	Last Updated: October 19, 2006
Medical Review:	Adam Husney, MD - Family Medicine Kathleen Romito, MD - Family Medicine Stephen Fort, MD, MRCP, FRCPC - Interventional Cardiology
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