儿童肥厚型心肌病临床分析

The clinical analysis of hypertrophic cardiomyopathy in children

目的探讨儿童肥厚型心肌病的临床特点、诊治方法及预后。方法对2000年10月至2013年9月在北京儿童医院确诊为肥厚型心肌病的25例患儿的临床表现、实验室检查、治疗方法及预后进行回顾性分析。结果肥厚型心肌病以年长儿童多见，平均年龄8岁9个月，男女比例3．1：1。临床表现：气促乏力5例，胸闷长出气4例，心前区不适3例，因咳喘就诊发现心脏异常5例，无症状发现心脏杂音来诊治8例，伴晕厥发作2例，6例有明确的肥厚型心肌病家族史，3例家族史中有幼年死亡病例（具体疾病不详）。心电图：18例提示左室和（或）右室肥厚伴ST—T改变，3例伴有异常Q波，2例有I。房室传导阻滞，1例有左前分支阻滞，1例有完全性左束支阻滞。心肌酶升高5例。X线胸片心影扩大17例。心脏彩超提示非梗阻型肥厚型心肌病23例；梗阻型肥厚型心肌病2例。收缩功能减低2例，舒张功能减低3例。21例患儿口服美托洛尔治疗，1例异搏定，1例心得安，未用药2例。随访2个月～13年，2例患儿死亡，其余23例生存。结论 小儿肥厚型心肌病临床表现缺乏特异性，病情重，呈进行性发展，是青少年猝死的主要原因之一，预后差。

Objective To investigate the clinical characteristics, diagnosis, treatment and prognosis of hypertrophic cardiomyopathy （HCM） in children. Methods Twenty-five cases of children confirmed with HCM in Beijing Children＇s Hospital from October 2000 to September 2013 were analyzed retrospectively through their clinical manifestations, laboratory tests, treatment and prognosis. Results HCM was found to be more common in older children. The average age of the patients in the studied cases was 8 years and 9 months and the male to female ratio was 3.1： 1. Their clinical manifestations were as the follows：5 cases with shortness of breath and fatigue ,4 cases with chest tightness and long breath ,3 cases with precordial discomfort, 5 cases with cough and wheezing and found to have cardiac abnormalities, 8 cases found with asymptomatic heart murmurs with no other symptom,2 cases with syncope episodes,6 cases with a clear HCM family history, 3 cases with a family history of infant deaths （ specific condition not clear）. ECG： 18 cases showing left ventricular and/or right ventricular hypertrophy and ST-T changes, 3 cases showing abnormal Q waves, 2 cases showing ST-T changes,2 cases with I ~ atrio-ventricular block, 1 case with left bundle branch block, and 1 case with left anterior division block. Cardiac enzymes were elevated in 5 cases. Chest X-rays showed enlarged heart shadow in 17 cases. Echocardiography revealed non-obstructive hypertrophic cardiomyopathy in 23 cases and obstructive hypertrophic cardiomyopathy in 2 cases. Left ventricular systolic function was de- creased in 2 cases, and diastolic dysfunction in 3 cases. Twenty-one patients took oral metoprolol, 1 patient took verapamil, 1 patient took propranolol and 2 patients took no medication. The follow-up period of 2 months to 13 years witnessed 2 cases of death and 23 cases of survival. Conclusion HCM in children lacks specific clinical manifestations and the conditions of the patient are usually severe with progressive development. HCM is one of the main reasons of sudden death in adolescent, with poor prognosis.