Restrictive cardiomyopathy is a heart disorder characterized by a primary decrease in ventricular compliance which results in poor ventricular filling during diastole. Unlike dilated cardiomyopathy, the contractile function of the left ventricle is usually preserved. Restrictive cardiomyopathy can be idiopathic or associated with several distinct diseases or processes that damage the myocardium including sarcoidosis, amyloidosis, postradiation fibrosis, endocardial fibroelastosis, Loffler’s syndrome and hemochromatosis. The ventricles in restrictive cardiomyopathy are usually of normal size or minimally enlarged and the myocardium is firm and noncompliant.
Sarcoidosis is characterized by the formation of numerous small granulomas in the heart wall and other tissues of the body. Involvement of the heart can decreased compliance and cause restrictive cardiomyopathy.
Amyloidosis is a progressive metabolic disease characterized by abnormal amyloid protein deposition in organs and body systems. Amyloid deposition in the heart can cause restrictive cardiomyopathy.
Postradiation fibrosis refers to scarring of organs including the heart resulting from overexposure to radiation, usually as a consequence from cancer therapy. Excessive scarring can decrease compliance of the heart leading to restrictive cardiomyopathy.
Endocardial fibroelastosis is a rare heart disease characterized by fibroelastic thickening that usually involves the left ventricular endocardium. It is most prevalent in the first 2 years of life and typically accompanied by obstruction of the aortic valve or other congenital cardiac anomalies.
Loeffler syndrome is a disease that occurs in response to a parasitic infection characterized by peripheral eosinophilia and eosinophilic infiltrates in other organs. Loeffler's syndrome can cause endomyocardial fibrosis with marked fibrosis of the ventricular endo and subendocardium that extends from the apex to the base of the heart. The fibrous tissue diminishes volume and compliance of affected chambers causing a restrictive cardiomyopathy.
Hemochromatosis is a disease characterized by excessive accumulation of iron in the body, most of which becomes deposited in organs such as the liver, pancreas, joints and heart. Deposition of hemosiderin in the heart can diminish compliance, causing a restrictive cardiomyopathy.
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