儿童扩张型心肌病临床特征分析

Clinical characteristics of pediatric dilated cardiomyopathy

目的了解儿童扩张型心肌病(DCM)临床特点和死亡相关因素。方法对2006—2011年115例DCM患儿临床资料进行整理分析。结果 115例患儿中,男70例(61%),女45例(39%);起病年龄1～180个月,平均(37.61±4.84)个月,起病≤1岁者占54%(62/115);初诊心功能Ⅲ～Ⅳ级者62.5%(72/115)。左室射血分数、左室缩短分数在心功能Ⅰ～Ⅱ级组[(33.0±7.9)%、(16.1±4.4)%]明显高于Ⅲ～Ⅳ级组[(29.2±7.5)%、(13.6±3.8)%](P<0.05);起病年龄≤1岁、～10岁、>10岁三组患儿经体表面积标准化的左房内径、左心室舒张末期内径、左心室收缩末期内径逐渐减小;Ⅲ～Ⅳ级组的cTnI、CK、CK-MB值均明显高于心功能Ⅰ～Ⅱ级组(P<0.05)。确诊时心功能级数(P<0.01)、左室射血分数(P<0.05)、左室缩短分数(P<0.05)是扩张型心肌病死亡相关因素。结论儿童扩张型心肌病确诊时≤1岁患儿最多,多为心功能Ⅲ～Ⅳ级,初诊年龄及心功能级别对患儿预后有明显影响;DCM患儿初诊心功能评估、超声心动图心功能参数可作为预测病情的指标。

objective To understand the clinical characteristics and death-related risk factors of pediatric dilated cardiomyopathy. Methods The clinical findings and follow-up data of 115 patients who were diagnosed during 2006-2011 in Shanghai Children＇s Medical Center were analyzed. Results In 115 patients, 70 were male （61%） and 45 were female （39%） with age of onset ranged from 1 to 180 months and average age of onset of （37.61±4.84） months. The age of onset in 54% cases of the patients were younger than one year, 62.5% cases of the patients had grade Ⅲ - Ⅳ of cardiac function at diagnosed. LVEF and LVFS in heart function grade Ⅰ-Ⅱgroup [（33.0±7.9） %, （16.1±4.4） %] was significantly higher than those in grade Ⅲ, Ⅳ group [（29.2±7.5） %, （13.6±3.8） %] （P〈 0.05）. LAD, LVDD and LVDSs tandardized by the body surface area were decreased with age in patients with different ages of onset （ ≤1 year, 1 to 10 years and ≥t 10 years, respectively）. The cTnI, CK, CK-MB values in cardiac function grade Ⅲ - IV group were significantly higher than those in cardiac function grade Ⅰ-Ⅱ group. Cardiac function at the time of diagnosis, LVEF, and LVFS were related to death in DCM patients. Conclusions At the time of definitive diagnosis, most patients were younger than 1 year and had cardiac function of grade Ⅲ - Ⅳ. It is suggested that age of onset and cardiac function grade have obvious influence in prognosis of patients; cardiac function evaluation at diagnosis and ultrasonic cardiogram