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Heart, section through the middle
Heart, section through the middle
Heart, front view
Heart, front view

Restrictive cardiomyopathy
Definition:

"Restrictive cardiomyopathy" referes to a group of disorders in which the heart chambers are unable to fill properly with blood because of stiffness of the heart. In restrictive cardiomyopathy, the heart is normal in size or only slightly enlarged, but it cannot relax normally during diastole (that is, the time between heartbeats in which the blood returns from the body to the heart).

Later in the disease, the heart may not pump blood efficiently. The abnormal heart function can affect the lungs, liver, and other body systems. Restrictive cardiomyopathy may affect either or both ventricles and may or may not be associated with a disease of the heartmuscle.
Alternative Names:
Cardiomyopathy - restrictive; Infiltrative cardiomyopathy

Causes, incidence, and risk factors:

The most common causes of restrictive cardiomyopathy are amyloidosis and idiopathic myocardial fibrosis (a scarring of the heart of unknown cause). Restrictive cardiomyopathy is frequently encountered after a heart transplantation. Other causes of restrictive cardiomyopathy include sarcoidosis, hemochromatosis, radiation fibrosis, and various tumor infiltrations of the heart. More rarely, restrictive cardiomyopathy is caused by diseases of the endocardium (the lining of the heart) such as endomyocardial fibrosis and Loeffler's syndrome.
Symptoms:
Signs and tests:
An examination may show signs of heart failure with fluid backup into the lungs or the systemic circulation (the extremities, gastrointestinal tract, and liver). The neck veins may be distended. Listening to the chest with a stethoscope (auscultation) may show lung crackles and may show abnormal or distant heart sounds.

Tests that may indicate restrictive cardiomyopathy (by showing symmetrical thickening of the ventricle walls and signs of abnormal heart function such as decreased cardiac output, and/or elevated end diastolic pressure) include:

Restrictive cardiomyopathy may be hard to differentiate from constrictive pericarditis. A biopsy of the heart muscle may be used to confirm the diagnosis. A cardiac catheterization procedure can sometimes help to differentiate the two cardiomyopathies by performing simultaneous left and right heart catheterization. In some cases, surgical exploration and biopsies are the only means to definitely distinguish restrictive cardiomyopathy from constrictive pericarditis.
Treatment:

Little therapy is known to be effective for the treatment of restrictive cardiomyopathy. The goal of treatment is to control symptoms and to improve the quality of life.

Various medications may be used to control symptoms. Diuretics may help somewhat in removing fluid, which can improve breathing. Depending on the underlying heart disease, some patients with restrictive cardiomyopathy may benefit from steroids or chemotherapy. A heart transplant may be considered if the function of the heart is very poor.
Expectations (prognosis):
People with restrictive cardiomyopathy may be candidates for heart transplant. Prognosis is dependent on the underlying etiology but it is usually poor. Average (mean) survival after diagnosis is 9 years.
Complications:
Progressive heart failure, mitral regurgitation, tricuspid regurgitation.
Calling your health care provider:
Call your health care provider if symptoms of restrictive cardiomyopathy are present.
Review Date: 5/9/2002
Reviewed By: Elena Sgarbossa, M.D., Department of Cardiology, Rush-Presbyterian St. Luke's Medical Ctr., Chicago, IL. Review provided by VeriMed Healthcare Network.
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