Restrictive cardiomyopathy

Outlined the characteristics of the type of primary cardiac and / or endocardial fibrosis, or myocardial invasive lesions, disruption caused cardiac filling the diastolic dysfunction. So far unknown etiology. In addition to invasive disease, the non-invasive nature of this type of cardiac disease pathogenesis research, focus on the eosinophil. the tropical and temperate regions seen some of the many patients with type eosinophil increase in the number. Early necrosis period, myocardial more eosinophils, generally within five weeks; Up to 10 months, endocardial thickening and thrombosis, myocardial reduce inflammation, thrombosis; two years after entering the period fibrosis, endocardial and myocardial fibrosis can be. Pathological changes in invasive disease caused by restrictive cardiomyopathy disease, amyloidosis (stroma amyloid accumulation), sarcoma (myocardial infiltration sarcoma-like substance), redness of the skin disease (myocardial containing hemosiderin deposition), glycogen accumulation disease (myocardial excessive accumulation of glycogen), and other types. In the non-invasive restrictive cardiomyopathy, endocardial myocardial fibers with Löffler endocarditis two species The former found in tropical and temperate found in the latter, the actual similarity. Heart appearance mildly or moderately increased significantly endocardial thickening and fibrosis, ventricular inflow tract and the apex of the main site, atrioventricular valve may also be affected, myocardial fibrosis can penetrate inside. Yi mural thrombus formation. Reduced ventricular cavity. Subendocardial myocardial may have calcification. Pathophysiological : endocardial and myocardial fibrosis occurred ventricular diastolic obstacles, but also associated with variable degree of systolic dysfunction. Ventricular cavity reduced, and thus ventricular filling restricted; Ventricle with a sharp decrease owners, there is an obstacle to the blood, subsequent cardiac output is also reduced, resulting in similar constrictive pericarditis the pathophysiological changes. Atrioventricular valve involvement can occur when the mitral or tricuspid regurgitation. As a result of the diagnosis of the clinical manifestations little early diagnosis more difficult. Clinical symptoms depended on the emergence of various checks to be confirmed, echocardiography is non-invasive and effective screening method. Subendocardial myocardial biopsy, if the specificity was found useful for the diagnosis, but also may be found infiltrates. Clinical with constrictive pericarditis identification, in particular the right ventricular disease mainly of restrictive cardiomyopathy, two similar clinical manifestations. In the history of acute pericarditis, pericardial X-ray showed calcification, chest CT or MRI scan showed pericardial thickening support pericarditis; ECG on atrial or ventricular hypertrophy, bundle branch block, systolic time interval is not normal support cardiomyopathy, echocardiography for the identification of the two will be helpful, apex chambers occlusion and heart intimal thickening established in the diagnosis of myocardial disease. For difficult cases, and for ventriculography endomyocardial biopsy. Clinical manifestations onset relatively slow. Early may have a fever and emerging weakness, dizziness, short breath. Lesions were mainly in the left ventricular and left ventricular failure and pulmonary hypertension performance of short breath, cough, hemoptysis, pulmonary rales base. Second pulmonary valve sound hyperthyroidism; Lesions in the right ventricle are mainly left ventricular blood to delay the performance of the jugular rolled, liver, lower extremity edema, ascites, and so on. Often weakened heart beat, voiced sector increased slightly, cardiophonogram light, fast heart rate, diastolic Benma a legal and arrhythmia. Pericardial effusion may also exist. Many see offal embolization. X-ray examination cardiac expansion may see subendocardial myocardial calcification of the shadow. Ventriculography see reduced ventricular cavity. ECG said low voltage, atrial or ventricular hypertrophy, bundle branch block, ST-T changes in atrial fibrillation, Also available on the V1, V2 leads with Q-wave abnormalities. Echocardiography Visibility endocardial thickening, apical ventricular cavity blocked, and subendocardial myocardial ultrasonic echo density anomaly, Kinesis weakened. In patients with primary wall thickening in the wall of invasive lesions can be thickened early diastolic filling fast, then slow later. General pericardial thickening. Cardiac catheterization said ventricular end-diastolic pressure increased gradually after the subsidence caused platform wave, predominantly in the left ventricular pulmonary artery pressure can be increased, mainly in the right ventricle were right atrial pressure, Right atrial pressure curve significant wave v to replace a wave. Systolic time interval measured not normal. Preventive treatment is limited to avoid complications. Not tired, to prevent infection. Mainly symptomatic treatment. Atrial fibrillation may have to digitalis; Have edema, and ascites it is appropriate diuretics. Application of diuretics or vasodilator should be careful not to allow ventricular filling pressure too much and affect cardiac function. To prevent embolization can be used anticoagulant drug. In recent years by excision fibrosis intimal thickening of the heart, atrioventricular valve impairment simultaneously artificial valve replacement surgery. can be better effect. Prognosis and course of mixed. Because the treatment is not complete, in the event of symptoms, which gradually lost labor, which eventually lead to death. Left ventricular lesions than the right ventricular lesions slightly after prognosis. In recent years, surgical treatment will bring hope.