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What Is Restrictive Cardiomyopathy?

Restrictive cardiomyopathy, the rarest form of cardiomyopathy, is a condition in which the walls of the lower chambers of the heart (the ventricles) are abnormally rigid and lack the flexibility to expand as the ventricles fill with blood.

The pumping or systolic function of the ventricle may be normal but the diastolic function (the ability of the heart to fill with blood) is abnormal. Therefore, it is harder for the ventricles to fill with blood, and with time, the heart loses the ability to pump blood properly, leading to heart failure.

What Are the Symptoms of Restrictive Cardiomyopathy?

Many people with restrictive cardiomyopathy have no symptoms or only minor symptoms, and live a normal life. Other people develop symptoms, which progress and worsen as heart function worsens.

Symptoms of restrictive cardiomyopathy can occur at any age and may include:

Less common symptoms of restrictive cardiomyopathy:

  • Fainting (usually caused by irregular heart rhythms or abnormal responses of the blood vessels during exercise)
  • Chest pain or pressure (occurs usually with exercise or physical activity but can also occur with rest or after meals)

Quick GuideHeart Disease: Symptoms, Signs, and Causes

Heart Disease: Symptoms, Signs, and Causes

Restrictive Cardiomyopathy Causes

What causes restrictive cardiomyopathy?

Restrictive cardiomyopathy is not usually inherited and its cause is often unknown. Known causes of restrictive cardiomyopathy may include:

  • Build-up of scar tissue (often for no known reason).
  • Build-up of abnormal proteins (amyloidosis) in the heart muscle.
  • Chemotherapy or chest exposure to radiation.
  • Excess iron (hemochromatosis) in the heart.
  • Other systemic diseases (sarcoidosis).

What Causes Restrictive Cardiomyopathy?

Restrictive cardiomyopathy is not usually inherited and its cause is often unknown. Known causes of restrictive cardiomyopathy may include:

How is Restrictive Cardiomyopathy Diagnosed?

The size of the heart may remain normal with restrictive cardiomyopathy. In some cases, restrictive cardiomyopathy may be confused with constrictive pericarditis, a condition in which the layers of the pericardium (the sac that surrounds the heart) become thickened, calcified, and stiff.

Restrictive cardiomyopathy is diagnosed based on medical history (your symptoms and family history), physical exam, and tests: such as blood tests, electrocardiogram, chest X-ray, echocardiogram, exercise stress test, cardiac catheterization, CT scan, and MRI.

A myocardial biopsy, or biopsy of the heart muscle, occasionally is done to determine the cause of cardiomyopathy. During a myocardial biopsy, a small tissue sample is taken from the heart and examined under a microscope to examine the cause of the symptoms.

What Is the Treatment for Restrictive Cardiomyopathy?

Treatment of restrictive cardiomyopathy is difficult. Treatment is usually focused on treating the cause of this condition. Doctors recommend lifestyle changes and medications to treat heart failure.

What Lifestyle Changes Are Recommended for Restrictive Cardiomyopathy?

Lifestyle changes can help restrictive cardiomyopathy. They might include:

Diet. Once you have symptoms such as shortness of breath or fatigue, you should restrict your intake of salt (sodium) to 2,000 milligrams to 3,000 milligrams per day. Follow this diet even when your symptoms abate.

Exercise. Your doctor will tell you if you may exercise or not. While exercise is generally good for the heart, people with this form of cardiomyopathy may experience fatigue and shortness of breath, even with minimal exertion. Therefore, experts recommend that you take frequent breaks, exercise at a time of day where you have the most energy and start slow, gradually building up strength and endurance. Heavy weight lifting is not recommended.

What Medications Are Used for Restrictive Cardiomyopathy?

Often, medications are used to treat symptoms of restrictive cardiomyopathy and prevent further complications. To manage heart failure, some people may improve by taking a beta-blocker and angiotensin-converting enzyme (ACE) inhibitor. If symptoms occur, digoxin, diuretics, and aldosterone inhibitors may be added. If you have an arrhythmia, your doctor may prescribe a medication to control your heart rate or lessen the occurrence of arrhythmia. Therapy may also be given to treat certain conditions, such as sarcoidosis, amyloidosis, and hemochromatosis. Your doctor will discuss what medications are best for you.

Quick GuideHeart Disease: Symptoms, Signs, and Causes

Heart Disease: Symptoms, Signs, and Causes

Can Surgery Treat Restrictive Cardiomyopathy?

In some cases, if the condition is severe, heart transplant surgery may be considered for restrictive cardiomyopathy.

WebMD Medical Reference

SOURCES:

Cardiomyopathy Association.

Reviewed by Thomas M. Maddox, MD on February 24, 2012

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Reviewed on 2/24/2012
References
SOURCES:

Cardiomyopathy Association.

Reviewed by Thomas M. Maddox, MD on February 24, 2012

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