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Ammula Uhanjali*, Kamalteja, Divi Manasa


Restrictive cardiomyopathy is represented by RCM.RCM is a rare form of heart muscle disease that is characterized by restrictive filling of the ventricles. This occurs because the heart muscle is stiff and does not allow the ventricular chambers to fill with blood normally. This inability to relax and fill with blood results in a back up of blood in to the atria, lungs and body causing signs and symptoms of heart failure. RCM is least common in children accounting 2-5%of diagnosed RCM .RCM affects girls some what more than boys. In most of cases the cause is unknown (idiopathic) although a genetic cause is suspected in most of pediatric RCM. Diagnosis is made by exercise intolerance, ECG, cardiacbiopsy, cardiac catherization, increased pulmonary artery pressure.. Diuretics, beta blockers and occasionally after load reducing agents are most common medications in RCM.Anti coagulant medications are needed in children if there is a risk of blood clots inside the heart possibly leading to a stroke. No surgery has been effective in improving the RCM. Heart transplantation is only effective treatment in those with RCM.A high calories, electrolytes rich diet is preferred in RCM. Children with RCM are not allowed to play sports for long time due to risk of sudden collapse, heart failure. Prognostic studies show 20% RCM patients have risk of thrombosis.

Keywords: RCM; Ventricular filling; Genetic cause; Heart transplantation.

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