A disorder of the myocardial musculature, restrictive cardiomyopathy is characterized by restricted ventricular filling (the result of left ventricular hypertrophy) and endocardial fibrosis and thickening. If severe, it's irreversible.
Restrictive cardiomyopathy is the least common form of cardiomyopathy. It has several similarities with hypertrophic cardiomyopathy. Restrictive cardiomyopathy is rare in the United States, Canada, and Europe. Other forms of include dilated cardiomyopathy, in which the heart muscle stretches out, and hypertrophic cardiomyopathy, in which the heart muscle is thicker than usual.
Primary restrictive cardiomyopathy is an extremely rare disorder of unknown cause. However, restrictive cardiomyopathy syndrome, a manifestation of amyloidosis, results from infiltration of amyloid into the intracellular spaces in the myocardium, endocardium, and subendocardium.
In both forms of restrictive cardiomyopathy, the myocardium becomes rigid, with poor distention during diastole, inhibiting complete ventricular filling. It fails to contract completely during systole, resulting in low cardiac output.
Signs and symptoms
Shortness of breath during exercise may be the first symptom you notice. As the condition progresses and heart failure develops, you may have difficulty breathing with even slight exertion. You may feel weak and tired. Many people have swollen legs from fluid retention (edema). A buildup of fluid around your liver, stomach, and intestines can cause bloating, nausea, and poor appetite.
Many people with primary restrictive cardiomyopathy first learn they have the disease when they develop complications, including stroke , heart attack , blood clot in the lung ( pulmonary embolism ), and other conditions related to blood clots
Although no therapy currently exists for restricted ventricular filling, digitalis glycosides, diuretics, and a restricted sodium diet ease the symptoms of heart failure.
Oral vasodilators-such as isosorbide dinitrate, prazosin, and hydralazine-may control intractable heart failure. Anticoagulant therapy may be necessary to prevent thrombophlebitis in the patient on prolonged bed rest.
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.
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