小儿肥厚型心肌病52例临床分析  

Clinical analysis of 52 cases of pediatric hypertrophic cardiomyopathy

作　　者：黄锡静 张丽[1] 梁小碧 陈燕[1] 汪周平[1] 谢小斐[1] 王燕飞 李伟[1] 袁家[1] 胡琳 黄萍[1] Huang Xijing;Zhang Li;Liang Xiaobi;Chen Yan;Wang Zhouping;Xie Xiaofei;Wang Yanfei;Li Wei;Yuan Jia;Hu Lin;Huang Ping(Department of Cardiology, Guangzhou Women and Children′s Medical Center Affiliated to Guangzhou Medical University, Guangzhou 510120, China)

机构地区：[1]广州医科大学附属广州市妇女儿童医疗中心心内科,510120

出　　处：《中华实用儿科临床杂志》2019年第16期1234-1236,共3页Chinese Journal of Applied Clinical Pediatrics

基　　金：广东省医学科研基金(A2018018).

摘　　要：目的总结小儿肥厚型心肌病临床特点,分析其病因,为早期识别、明确诊断和判断预后提供指导。方法回顾性分析2012年1月至2018年6月广州市妇女儿童医疗中心收治的52例肥厚型心肌病患儿,从性别、家族史、临床特征、辅助检查、病因学、药物疗效、病情转归等方面进行总结。结果本组病例共52例,其中男36例,女16例。随访34例(随访率65.4%),随访1~78个月(平均30.6个月)。超声心动图提示左室壁增厚52例(100.0%)、双心室肥大21例(40.4%)、左心室流出道梗阻18例(34.6%)。11例患儿病因明确(21.2%),其中Ⅱ型糖原累积病7例,Noonan综合征3例,原发性肉碱缺乏1例。11例患儿曾使用β受体阻滞剂治疗,均有不同程度的症状缓解。至随访终点无一例行心脏移植,死亡12例(35.3%),其中7例于婴儿期死亡。结论小儿肥厚型心肌病发病年龄较小,应积极寻找病因,完善疾病风险评估,进行个性化管理和治疗。Objective To summarize the clinical characteristics of pediatric hypertrophic cardiomyopathy and analyze its etiology for providing guidance for early identification, diagnosis and prognosis. Methods Fifty-two cases of pediatric hypertrophic cardiomyopathy admitted to Guangzhou Women and Children′s Medical Center from January 2012 to June 2018 were retrospectively analyzed and summarized from the aspects of age, gender, family history, clinical features, auxiliary examination, etiology, drug efficacy and disease outcome. Results (1) There were 52 cases in this group including 36 males and 16 females.The ages of patients ranged from 15 days to 14 years (with mean age of 27.7 months, median 6.5 months). A total of 34 patients (65.4%) were followed up for 1-78 months (mean 30.6 months). Echocardiography showed 52 cases of left ventricular wall thickening (100%), 21 cases of double ventricular hypertrophy (40.4%), 18 cases of left ventricular outflow tract obstruction (34.6%), and 18 cases of hepatic enzyme elevation (34.6%). The etiology of 11 cases was clear (21.2%), including 7 cases of type Ⅱ glycogen accumulation, 3 cases of Noonan syndrome and 1 case of primary carnation deficiency.No routine heart transplantation was performed at the end of follow-up, and 12 patients (35.3%) died, 7 cases of whom died in infancy. Conclusions Children with hypertrophic cardiomyopathy have a relatively young age, so it is necessary to search for the etiology actively, carry out disease risk assessment, and conduct personalized management and treatment.

关 键 词：小儿 心肌病 肥厚型 

分 类 号：R725.4[医药卫生—儿科]