CAJ | 학술논문

目的了解儿童扩张型心肌病(DCM)的病因、临床特点及预后相关因素.方法对2008年1月至2012年12月183例DCM患儿住院及随访资料进行分析.结果 183例患儿中,24例(13.1％)病因明确;病因不明的随访病例共132例,其中男85例(64.0％),女47例(36.0％);恢复33例(25.0％),死亡62例(47.0％),无变化者37例(28.0％).中位起病年龄为8个月(1 d～15岁).恢复组起病年龄小于死亡组(P＜0.001)与无变化组(P =0.008).死亡组初诊时心功能差于恢复组(P ＜0.001)与无变化组(P=0.015);心脏彩超(UCG)所测左心室射血分数(LVEF)、左心室短轴缩短率(LVFS)低于恢复组(P =0.006、0.009)和无变化组(P=0.013、0.006),中、重度二尖瓣反流(MR)者多于恢复组(P--0.003)和无变化组(P =0.004).无变化组血清肌钙蛋白Ⅰ水平≥0.10 μg/L者少于恢复组与死亡组(P=0.002、0.004).病因未明的159例病例,1年生存率67％,5年生存率46％.LVFS[HR0.890,95％可信区间(CI)0.836 ～0.948,P＜0.001],中重度MR(HR3.580,95％ CI 2.030～6.315,P＜0.001),室性恶性心律失常(HR2.062,95％ CI1.009 ～4.213,P=0.047),异常Q波(HR4.044,95％ CI1.851 ～8.832,P＜0.001)是死亡危险因素.结论病因不明的儿童原发性DCM经适当治疗25.0％可获临床痊愈,近50.0％死亡;1年生存率67.0％,5年生存率46.0％.起病后3个月左心室功能改善明显者,预后较好.初诊时低LVFS,二尖瓣中-重度反流,存在室性恶性心律失常、异常Q波是死亡危险因素.
목적 료해인동확장형심기병(DCM)적병인、림상특점급예후상관인소.방법 대2008년1월지2012년12월183례DCM환인주원급수방자료진행분석.결과 183례환인중,24례(13.1％)병인명학;병인불명적수방병례공132례,기중남85례(64.0％),녀47례(36.0％);회복33례(25.0％),사망62례(47.0％),무변화자37례(28.0％).중위기병년령위8개월(1 d～15세).회복조기병년령소우사망조(P＜0.001)여무변화조(P =0.008).사망조초진시심공능차우회복조(P ＜0.001)여무변화조(P=0.015);심장채초(UCG)소측좌심실사혈분수(LVEF)、좌심실단축축단솔(LVFS)저우회복조(P =0.006、0.009)화무변화조(P=0.013、0.006),중、중도이첨판반류(MR)자다우회복조(P--0.003)화무변화조(P =0.004).무변화조혈청기개단백Ⅰ수평≥0.10 μg/L자소우회복조여사망조(P=0.002、0.004).병인미명적159례병례,1년생존솔67％,5년생존솔46％.LVFS[HR0.890,95％가신구간(CI)0.836 ～0.948,P＜0.001],중중도MR(HR3.580,95％ CI 2.030～6.315,P＜0.001),실성악성심률실상(HR2.062,95％ CI1.009 ～4.213,P=0.047),이상Q파(HR4.044,95％ CI1.851 ～8.832,P＜0.001)시사망위험인소.결론 병인불명적인동원발성DCM경괄당치료25.0％가획림상전유,근50.0％사망;1년생존솔67.0％,5년생존솔46.0％.기병후3개월좌심실공능개선명현자,예후교호.초진시저LVFS,이첨판중-중도반류,존재실성악성심률실상、이상Q파시사망위험인소.
Objective To explore the etiology,clinical features and outcomes predictors of pediatric dilated cardiomyopathy(DCM).Methods The clinical findings and follow-up data of 183 pediatric DCM patients who were diagnosed during Jan.2008 to Dec.2012 were analyzed.Results In the 183 patients,the cause of 24 cases (13.1％)was known;but the etiology of 132 follow-up cases remain unknown,85 were male (64.0％) and 47 were female (36.0％) ;33 cases (25.0％) recovered,62 cases (47.0％) died,and 37 cases (28.0％) remained the same.The median age of onset was 8 months(ranged from 1 day to 15 years).The onset age of the patients who had recovered was younger than the dead (P ＜ 0.001) and the unchanged patients (P =0.008).For the dead cases,the heart function on diagnosis was worse than the cured(P ＜ 0.001) and the unchanged patients (P =0.015) ;the left ventricular ejection fraction(LVEF) and the left ventricular fractional shortening(LVFS) detected by ultrasonic cardiogram were significantly lower than those of the cured(P =0.006,0.009) and the unchanged (P =0.013,0.006) ;the cases with moderate and serious mitral regurgitation(MR) were also more than the other groups (P =0.003,0.004) ;The cases in the unchanged group were less than those of the cured group and the dead group whose serum concentration of cardiac troponin I was≥0.10 μg/L(P =0.002,0.004).For the 159 cases with the unknown cause,1-year survival rate was 67 ％ and 5-year survival rate was 46 ％.By multivariable analysis,LVFS [hazard ratios (HR) 0.890,95 ％ confidence interval (CI) 0.836-0.948,P ＜ 0.001],moderate and serious MR [HR 3.580,95％ CI 2.030-6.315,P ＜ 0.001],malignant ventricular arrhythmia [HR 2.062,95 ％ CI 1.009-4.213,P =0.047],abnormal Q wave [HR 4.044,95 ％ CI 1.851-8.832,P ＜ 0.001] were associated with increased risk of death.Conclusions Twenty-five percent pediatric idiopathic DCM patients with the unknown cause recovered after proper treatment,and nearly 50.0％ patients died;1-year survival rate was 67.0％ and 5-year survival rate was 46.0％.Three months after onset,the patients whose left ventricular function improved obviously had a better outcome.Risk factors for death include LVFS,moderate and serious MR,malignant ventricular arrhythmia,abnormal Q wave.