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Terms in this set (12)
Restrictive cardiomyopathy refers to changes in and weakening of the heart muscIe, which causes the heart to fill poorly, squeeze poorly, or both. In restrictive cardiomyopathy, the heart is of normal size or only slightly enlarged. However, it cannot relax normally during the time between heartbeats when the blood returns from the body (diastole). Later in the disease, the heart may not pump blood strongly. The abnormal heart function can affect the lungs, liver, and other body systems. Restrictive cardiomyopathy may affect either or both ventricles. It is usually associated with a disease of the heart muscle.
Restrictive cardiomyopathy Causes
Although restrictive cardiomyopathy is a rare condition, the most common causes are amyloidosis and scarring of the heart from an unknown cause (idiopathic myocardial fibrosis). It can occur after a heart transplant.
Other causes of restrictive cardiomyopathy include:
Carcinoid heart disease
Diseases of the heart lining (endocardium), such as endomyocardial fibrosis and Loeffler's syndrome (rare)
Iron overload (hemochromatosis)
Scarring after radiation or chemotherapy
Tumors of the heart
Symptoms of heart failure are most common. Usually, these symptoms develop slowly over time. However, sometimes symptoms start very suddenly and are severe.
Common symptoms are:
Difficulty breathing or shortness of breath
- Especially with activity
- When lying flat
Fatigue, poor exercise tolerance Loss of appetite
Swelling of the abdomen
Swelling of the feet and ankles
Uneven or rapid pulse
Other symptoms may include:
Decreased alertness or concentration
Low urine production
Need to urinate at night (in adults)
Exams and Tests
An examination may show:
Enlarged (distended) or bulging neck veins
Lung crackles and abnormal or distant heart sounds when listening to the chest with a stethoscope
Signs of heart failure
Fluid backup into the hands and feet
Tests for restrictive cardiomyopathy include:
Cardiac catheterization and coronary angiography
Chest CT scan
Echocardiogram and Doppler study
MRI of the heart Nuclear heart scan (MUGA, RNV)
Serum iron studies
Serum or urine protein tests
Restrictive cardiomyopathy may be hard to tell apart from constrictive pericarditis. A biopsy of the heart muscle or cardiac catheterization may help confirm the diagnosis, but is not often done.
When the cause of any cardiomyopathy can be found, that condition is treated. Few treatments are known to be effective for restrictive cardiomyopathy. The main goal of treatment is to control symptoms and improve quality of life.
The following treatments may be used to control symptoms or prevent problems:
Blood thinning medications, either aspirin or warfarin Chemotherapy (in some situations)
Diuretics to remove fluid and help improve breathing Medications to prevent or control uneven or abnormal heart rhythms
Steroids for some causes
A heart transplant may be considered if the heart function is very poor and the patient has severe symptoms.
People with this condition often develop heart failure that gets worse. Problems with "leaky" heart valves may also occur.
People with restrictive cardiomyopathy may be heart transplant candidates. The outlook depends on the cause of the condition, but it is usually poor. Average survival after diagnosis is 9 years.
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