Disease Information for Cardiomyopathy, restrictive: Definition

  • Myocardial disease in which the ventricular walls are excessively rigid, impeding ventricular filling; it is marked by abnormal diastolic function but by normal or nearly normal systolic function---------------------------- infitrative types of pathology include Amyloid, Hemochramotosis, Glycogen storage, Scleroderma,Fibrosis, Sarcoidosis;---Cardiomyopathy, Restrictive Fred F Ferri MD

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    Cardiomyopathies are a group of diseases primarily involving the myocardium and characterized by myocardial dysfunction that is not the result of hypertension, coronary atherosclerosis, valvular dysfunction, or pericardial abnormalities; Restrictive cardiomyopathies are characterized by decreased ventricular compliance, usually secondary to infiltration of the myocardium; Relatively uncommon cardiomyopathy that is most frequently caused by amyloidosis , myocardial fibrosis (after open heart surgery), and radiation; Edema, ascites, hepatomegaly, distended neck veins, Fatigue, weakness (secondary to low output); Kussmaul’s sign: may be present; Regurgitant murmurs; Possible prominent apical impulse; Infiltrative and storage disorders (glycogen storage disease, amyloidosis, sarcoidosis, hemochromatosis) and also Scleroderma; Radiation; Endocardial fibroelastosis;

    Endomyocardial fibrosis; Idiopathic, Hypereosinophilic syndromes; Toxic effects of anthracycline; Carcinoid heart disease, metastatic cancers; Diabetic cardiomyopathy

    Eosinophilic cardiomyopathy (Löffler’s endocarditis)

    DIAGNOSIS: Chest x-ray examination, ECG, echocardiogram

    Cardiac catheterization, MRI (selected cases);Chest x-ray examination: Moderate cardiomegaly;Possible evidence of CHF (pulmonary vascular congestion, pleural effusion);ECG:

    Low voltage with ST-T wave changes; Possible frequent arrhythmias, left axis deviation, and atrial fibrillation

    Echocardiogram: increased wall thickness and thickened cardiac valves (especially in patients with amyloidosis)

    Cardiac catheterization to distinguish restrictive cardiomyopathy from constrictive pericarditis; Constrictive pericarditis: usually involves both ventricles and produces a plateau of elevated filling pressures; Restrictive cardiomyopathy: impairs the left ventricle more than the right (PCWP > RAP, PASP >50 mm Hg) MRI may also be useful to distinguish restrictive cardiomyopathy from constrictive pericarditis (thickness of the pericardium >5 mm in the latter) Treatment includes : Sodium restriction, diuretics, ACE inhibitors, β-blockers, spironolactone, and digitalis;

    Vasodilators (combined with nitrates and ACE inhibitors) are effective agents in all symptomatic patients with left ventricular dysfunction; Prevent thromboembolism with oral anticoagulants in all patients with atrial fibrillation and in patients with moderate or severe failure; Low-dose β-blockade with carvedilol or other β-blockers may improve ventricular function by interrupting the cycle of reflex sympathetic activity and controlling tachycardia--------

    [merck medicus website ferri clinical advisor 2005]---------