Restrictive cardiomyopathy

Definition

Cardiomyopathy is an ongoing disease process that damages the muscle wall of the lower chambers of the heart. Restrictive cardiomyopathy is a form of cardiomyopathy in which the walls of the heart become rigid.

Description

Restrictive cardiomyopathy is the least common type of cardiomyopathy in the United States. The stiffened heart walls cannot stretch properly to allow enough blood to fill the ventricles between heartbeats. As the stiffening worsens, heart failure occurs. The blood backs up into the blood vessels, causing fluid buildup in tissues (congestion and edema).

Causes & symptoms

Restrictive cardiomyopathy can be caused by a number of diseases. Often, the cause is unknown. The rigidity of the heart walls may be caused by fibrosis, the replacement of muscle cells with tough, fibrous tissue. In some disorders, proteins and other substances are deposited in the heart wall. Amyloidosis is the accumulation of a protein material, called amyloid, in the tissue of the heart wall and other organs. In hemochromatosis, there is too much iron in the body and some of the excess iron can build up in the heart. Sarcoidosis causes the formation of many small lesions, called granulomas, in the heart wall and other tissues of the body. These granulomas contain inflammatory white blood cells and other cells that decrease the flexibility of the heart.

People with restrictive cardiomyopathy usually feel tired and weak, and have shortness of breath, especially during exercise. If blood is backing up in the circulation they may also experience edema (large amounts of fluid in tissues) of the legs and feet.

Diagnosis

The diagnosis is usually based on a physical examination, echocardiography, and other tests as needed. The physician listens to the heart with a stethoscope to detect abnormal heart rhythms and heart sounds.

Echocardiography uses sound waves to make images of the heart. These images provide information about the structures of the heart and its heart valves. Echocardiography can also be used to find out how much blood the heart is pumping. It determines the amount of blood in the ventricle, called the ventricular volume, and the amount of blood the ventricle pumps each time it beats, called the ejection fraction. A healthy heart pumps at least one half the amount of blood in the left ventricle with each heartbeat.

Computed tomography scan (CT scan) and magnetic resonance imaging (MRI) are imaging tests that can also provide information about the structure of the heart. However, these tests are rarely needed for diagnosis.

Cardiac catheterization may be needed to confirm a diagnosis or cause. In cardiac catheterization, a small tube called a catheter is inserted into an artery and passed into the heart. It is used to measure pressure in the heart and the amount of blood pumped by the heart. A small tissue sample (biopsy) of the heart muscle can be removed through the catheter for microscopic examination. Fibrous tissue or deposits in the heart muscle can be identified in this biopsy.

Treatment

There is no effective treatment for restrictive cardiomyopathy. Treatment of a causative disease may reduce or stop the damage to the heart, but existing damage cannot be reversed. Medications may be used to lessen the workload on the heart and to control the heart rhythm. Drugs normally used to treat other types of cardiomyopathy and heart failure may cause problems for patients with restrictive cardiomyopathy. For example, medicines that reduce the heart's workload may lower blood pressure too much.

A heart transplant may be necessary for patients who develop severe heart failure.

Prognosis

The prognosis for patients with restrictive cardiomyopathy is poor. If the disease process causing the problem can be treated, the damage to the heart muscle may be stopped. Also, medicines may relieve symptoms. However, for most patients, restrictive cardiomyopathy eventually causes heart failure. A heart transplant may be necessary when heart failure becomes too severe to treat with medicines.

Prevention

Obtaining early treatment for diseases that might cause restrictive cardiomyopathy might prevent or slow the development of heart wall stiffness. Anyone experiencing symptoms of shortness of breath, tiredness, and weakness should see a physician.

Key Terms

Amyloidosis

Build up of amyloid, a protein substance, in tissues of the body, including the heart.

Cardiac catheterization

A diagnostic test for evaluating heart disease; a catheter is inserted into an artery and passed into the heart.

Edema

Swelling caused by fluid buildup in tissues.

Fibrosis

Replacement of normal tissue with tough, fibrous tissue.

Hemochromatosis

A disease in which there is too much iron in the body; iron deposits can build up in the heart muscle and other tissues.

Sarcoidosis

A chronic disease causing the formation of many small lesions called granulomas in the heart wall and other tissues of the body.

Further Reading

For Your Information

Books

• Bellenir, Karen, and Peter D. Dresser, editors. Cardiovascular Diseases and Disorders Sourcebook. Detroit, MI: Omnigraphics, 1995.
• Texas Heart Institute. Heart Owner's Handbook. New York: John Wiley and Sons, 1996.

  Organizations

• American Heart Association. 7272 Greenview Avenue, Dallas, TX 75231-4596. (800) AHS-USA1. http://www.amhrt.org/.
• National Heart, Lung, and Blood Institute. Information Center, PO Box 30105, Bethesda, MD 20824-0105. (301) 251-1222.
• Texas Heart Institute. Heart Information Service, PO Box 20345, Houston, TX 77225-0345. (800) 292-2221.

Gale Encyclopedia of Medicine. Gale Research, 1999.