原发性扩张型心肌病96例患儿长期随访  被引量:4

Long Term Follow-Up for 96 Children with Primary Dilated Cardiomyopathy

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作  者:段庆宁[1] 王凤鸣[2] 赵乃峥[2] 曹黎明[2] 沙红[1] 李琪[1] 李红[1] 

机构地区:[1]南京医科大学附属无锡市人民医院儿科,江苏无锡214023 [2]南京医科大学附属南京儿童医院心血管内科,南京210008

出  处:《实用儿科临床杂志》2011年第13期997-1000,共4页Journal of Applied Clinical Pediatrics

摘  要:目的探讨原发性扩张型心肌病(DCM)患儿临床特点、长期预后及其死亡的相关危险因素。方法对1985年10月-2008年10月南京医科大学附属南京儿童医院收住院的118例DCM患儿进行随访。118例DCM患儿中共96例(81%)获得随访,22例(19%)失访。根据患儿转归不同将96例获得随访的患儿分为死亡组(53例)和存活组(43例)。应用SPSS 13.0软件进行统计学分析。结果 DCM患儿1 a、2 a、3 a和5 a生存率分别为65.60%、62.50%、56.25%及44.79%。性别、家族史、心力衰竭史及奔马律在死亡组和存活组间差异均无统计学意义,而农村人口(死亡组35例vs存活组18例,P=0.018)、初次发病平均年龄(死亡组M 35.3个月,Q 8.4~99.0个月;存活组M 13.7个月,Q 5.5~42.3个月,P=0.002)及从发病到就诊平均时间(死亡组M 21.0 d,Q 9.5~60.0 d;存活组M 14.0 d,Q 7.0~21.0 d,P=0.043)在二组间差异有统计学意义。ECG示左心室肥厚(死亡组23例vs存活组30例,P=0.010)可能是预后良好的指标,而心房扩大(死亡组14例vs存活组2例,P=0.005)及病理性Q波(死亡组7例vs存活组0例,P=0.022)往往提示预后不良。心脏超声中EF值≤20%(死亡组13例vs存活组0例,P=0.001)、FS值≤10%(死亡组16例vs存活组4例,P=0.012)及治疗后EF和(或)FS增长率不明显[死亡组4.14%和(或)5.53%vs存活组55.48%和(或)63.62%,Pa=0.000]提示预后不佳。初次发病年龄〉5岁患儿病死率显著高于发病年龄≤5岁者(76.92%vs47.14%,P=0.009)。初次发病年龄〉5岁(OR=16.000,95%CI2.001~127.925,P=0.009)是小儿DCM独立危险因素,左心室肥厚(OR=0.057,95%CI0.014~0.222,P=0.000)则是独立保护性因素。结论 DCM常预后不良,远期存活率不高,发病年龄〉5岁是小儿DCM的独立危险因素。Objective To investigate the clinical characteristics,long term prognosis and risk factors for death in children with primary dilated cardiomyopathy(DCM).Methods Clinical and follow-up data from 118 children with DCM hospitalized in Nanjing Children′s Hospital Affiliated to Nanjing Medical University from Oct.1985 to Oct.2008 were retrospectively analyzed.A total of 96 out of 118 patients(81%) were followed-up.The 96 cases of children were divided into 2 groups according to their outcomes,including death group(n=53) and survive group(n=43).The data were analyzed by SPSS 13.0 software.Results The survival rates of all followed-up children at 1 year,2 years,3 years and 5 years were 65.60%,62.50%,56.25% and 44.79%,respectively.Single risk factor analysis indicated that the gender,family history,history of heart failure and gallop rhythm had no significant difference,countryman(death group 35 cases vs survival group 18 cases,P=0.018),age at presentation(death group M 35.3 months,Q 8.4-99.0 months;survive group M 13.7 months,Q 5.5-42.3 months,P=0.002) and the average time from illness onset to diagnosis in death group M 21.0 days,Q 9.5-60.0 days;survival group M 14.0 days,Q 7.0-21.0 days(P=0.043) had significant differences between 2 groups.Left ventricular hypertrophy(23 cases vs 30 cases) was correlated with better prognosis.Enlargement of atrium,and Q wave in ECG(14 cases vs 2 cases;7 cases vs 0) were correlated with poor prognosis.The left ventricular ejection fraction(EF) ≤20% in Doppler echocardiography(13 cases vs 0,P=0.001),the fractional shortening(FS) ≤10%(16 cases vs 4 cases,P=0.012) and failure to increase EF or/and FS from presentation(death group 4.14% and/or 5.53% vs survival group 55.48% and/or 63.62%,P=0.000) were associated with worse outcomes.The children′s DCM onset5 years of age had worse outcomes than the onset ≤5 years of age(death rate 76.92% vs 47.14%,P=0.009).By multivariate analysis,age 5 years at pesentation(OR=16.000,9

关 键 词:扩张型心肌病 原发性 随访 预后 危险因素 儿童 

分 类 号:R725.4[医药卫生—儿科]

 

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