Restrictive cardiomyopathy A “stiff” heart with reduced filling in diastole Dilated atria Endomyocardial fibrosis (EMF,tropical) Subendocardial fibrosis with thrombosis Loeffler endomyocarditis Similar to EMF but with eosinophil infiltrate (possibly related to parasite infection) Amyloid heart disease has similar features
18 Definition of RCM: Restrictive cardiomyopathy refers to a group of disorders in which the heart chambers are unable to fill with blood properly 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 heart muscle.
19 Causes The most common causes of restrictive cardiomyopathy are amyloidosis and idiopathic myocardial fibrosis (a scarring of the heart of unknown cause). It frequently occurs after a heart transplant. 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 such as endomyocardial fibrosis and Loeffler's syndrome.
20 Symptoms Excessive tiredness (fatigue). Swelling of the feet and ankles. Cough. Difficulty breathing: – especially with exertion – at night – when lying flat Easily fatigued (poor tolerance of exercise). Swelling of the abdomen.
21 Diagnosis : Restrictive cardiomyopathy may be hard to differentiate from constrictive pericarditis. A biopsy of the heart muscle may be used to confirm the diagnosis.
22 Prognosis: People with restrictive cardiomyopathy may be candidates for heart transplant. Prognosis is dependent on the underlying cause but it is usually poor. Average (mean) survival after diagnosis is 9 years.
23 Possible Complications: Progressive heart failure. Mitral regurgitation. Tricuspid regurgitation.
24 Endomyocardial fibrosis:Histologic Findings: The heart size is not usually enlarged in EMF. The ventricular cavities are frequently laden with thrombi and, in severe cases, may be nearly totally obliterated by endocardial thickening and thrombosis. The histologic findings of EMF are characterized by reactive fibrosis associated with a selective increase in type I collagen deposition, subendocardial infarction and fibrosis, and thrombus formation. Additionally, specific features of other diseases, such as those associated with hemochromatosis or glycogen storage disease, are notably absent.
25 RCM due to hemochromatosis: Prussian Blue reaction shows iron deposits in myocardium.
26 Restrictive cardiomyopathy with amyloidosis