Yibian
 Shen Yaozi 
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diseasePediatric Restrictive Cardiomyopathy
aliasRestrictive Cardiomyopathy
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bubble_chart Overview

Restrictive cardiomyopathy primarily occurs in tropical and subtropical regions, including Africa, South Asia, and South America. It is very rare in China, with all reported cases found in the southern regions, showing a sporadic distribution. The disease mainly involves fibrosis of the endocardium and myocardium, leading to impaired ventricular contraction and relaxation, reduced ventricular cavity size, restricted ventricular filling, decreased ventricular compliance, hindered blood return, and reduced cardiac output. These pathological and physiological changes resemble those seen in constrictive pericarditis.

bubble_chart Diagnosis

(1) Clinical Manifestations The onset is relatively slow, with symptoms such as lack of strength, palpitation, and dyspnea. In cases where the right ventricle is predominantly affected, manifestations of right ventricular tamponade may occur, including jugular vein distension, hepatomegaly, lower limb edema, and ascites. In cases where the left ventricle is predominantly affected, symptoms such as shortness of breath, cough, hemoptysis, pulmonary rales, and accentuated pulmonary stirred pulse second heart sound may be observed. Cardiac impulse is weak, the dullness border is grade I enlarged, heart sounds are faint, heart rate is rapid, and diastolic gallop rhythm, arrhythmia, or signs of visceral embolism may be present.

(2) X-ray Examination Findings may include mild to grade II enlargement of the cardiac silhouette, possible calcification spots in the endocardial membrane, weakened cardiac pulsation, and pulmonary congestion.

(3) Electrocardiogram (ECG) Changes such as ST-T abnormalities, ventricular hypertrophy, and bundle branch block may be observed.

(4) Echocardiography Two-dimensional echocardiography may reveal reduced cardiac chamber size, thickened endocardial membrane, and weakened ventricular wall motion. Abnormalities in systolic time intervals may also be detected. Clinically, differentiation from constrictive pericarditis can sometimes be challenging. If necessary, heart blood vessel angiography and endocardial membrane myocardial biopsy may be performed to confirm the diagnosis.

bubble_chart Treatment Measures

﹝Treatment﹞

is mainly symptomatic. Diuretics can be used for those with edema and ascites, and anticoagulants may be administered to prevent embolism. Recently, some have performed surgical resection of the thickened endocardium, and for those with affected atrioventricular valves, simultaneous artificial valve membrane replacement has shown some effectiveness.

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