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小儿原发性心肌病为严重危害小儿健康的心脏疾患,愈后差、病死率高,早期诊断治疗对改善预后至关重要。现将我科1984～1997年收治的原发性心肌病30例作一分析。 1 临床资料 1.1 一般资料 30例均符合全国九省市心肌炎协作组制定的小儿心肌病诊断标准。男19例,女11例。年龄2个月至14岁(平均7.3岁)。30例中,扩张型24例,肥厚型5例,限制型1例。 1.2 临床表现 首诊时病程12天至6年,病程半年以内19例,半年到1年3例,1年以上者8例。24例扩张型心肌病以心脏扩大伴心衰表现而就诊18例,以面色苍白,胸闷气促入院17例,因水肿而误诊为肾病综合征入院2例,以肺炎并心衰入院6例,均为婴幼儿。5例肥厚型心肌病有呼吸困难表现4例,胸痛3例,疲乏3例,晕厥2例,1例有家族史。限制型心肌病1例以双下肢水肿3个月入院。
Primary cardiomyopathy in children as a serious harm to children’s heart disease, poor prognosis, high mortality, early diagnosis and treatment to improve prognosis is essential. Now our department admitted from 1984 to 1997, 30 cases of primary cardiomyopathy for an analysis. 1 Clinical data 1.1 General Information 30 cases are in line with the nine provinces of myocarditis collaborative group developed diagnostic criteria for pediatric cardiomyopathy. 19 males and 11 females. Ages 2 months to 14 years (average 7.3 years). Of the 30 patients, 24 were dilated, 5 were hypertrophic, and 1 was restricted. 1.2 The first clinical manifestations course of 12 days to 6 years, duration of less than six months in 19 cases, six months to 1 year in 3 cases, more than 1 year in 8 cases. Twenty-four patients with dilated cardiomyopathy underwent cardiac enlargement and heart failure. Of the 18 patients, pale, chest tightness and shortness of breath were admitted to the hospital, 17 were admitted to hospital due to edema, 2 were admitted to hospital with nephrotic syndrome, 6 were admitted to hospital with pneumonia and heart failure, Are infants and young children. Five patients with hypertrophic cardiomyopathy had dyspnea in 4 cases, 3 cases of chest pain, 3 cases of fatigue, 2 cases of syncope, and 1 case of family history. One case of restrictive cardiomyopathy was admitted to hospital for three months with edema of both lower extremities.