儿童左心室肥厚的临床病因分析

Etiological analysis of left ventricular hypertrophy in children

目的了解儿童左心室肥厚(LVH)的病因分布。方法对2005年3月至2008年3月在广州市儿童医院二维超声诊断LVH的27例患儿(男19例、女8例,年龄平均2.66岁)行病因诊断分析。结果 27例LVH中主动脉病变所致4例(14.8%),糖原累积病Ⅱ型所致2例(7.4%),肢端肥大症所致1例(3.7%),黏多糖蓄积症所致1例(3.7%);6例(22.2%)有家族史且无明显临床症状者考虑为肥厚型心肌病(HCM);病因未明占13例(48.1%),须进一步跟踪分析。结论心脏影像学检查提示LVH的患儿病因复杂多样,不能简单根据影像学的结果诊断HCM,进一步完善LVH病因的诊断程序,区分儿童期及成人期LVH病因分布是必要的。

Objective Left ventricular hypertrophy（LVH）,especially in children,is associated with a series of disorder including cardiac and systemic diseases.The etiological diagnosis should be evaluated in pediatric LVH patients.Methods From March 2005 to March 2008,clinical data from 27 patients（19 males and 8 females,mean age 2.66 years）with LVH diagnosed by two-dimensional ultrasound were analyzed.Results Among 27 patients,the primary disease were aortic disease in 4（14.8%）patients,glycogen-storage disease in 2（7.4%）patients,acromegaly in one（3.7%）patient,and glycosaminoglycan disease in one（3.7%）patient.Six（22.2%）patients with positive family history of LVH were considered to have hypertrophic cardiomyopathy.The primary diseases were unclear in another 13（48.1%）patients.Conclusions The primary diseases were complicated in LVH patients diagnosed by imaging examination.The diagnosis of LVH should be not only based on imaging examination,but also relied on etiological diagnosis.