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心肌炎患儿的心室重构和存活率优于特发性扩张型心肌病患儿:来自儿科心肌病注册中心的一项结局研究。

Ventricular remodeling and survival are more favorable for myocarditis than for idiopathic dilated cardiomyopathy in childhood: an outcomes study from the Pediatric Cardiomyopathy Registry.

机构信息

Washington University at St Louis, 1 Children’s Place, St Louis, MO 63110, USA.

出版信息

Circ Heart Fail. 2010 Nov;3(6):689-97. doi: 10.1161/CIRCHEARTFAILURE.109.902833. Epub 2010 Sep 10.

DOI:10.1161/CIRCHEARTFAILURE.109.902833
PMID:20833772
Abstract

BACKGROUND

Myocarditis is a cause of a new-onset dilated cardiomyopathy phenotype in children, with small studies reporting high rates of recovery of left ventricular (LV) function.

METHODS AND RESULTS

The presenting characteristics and outcomes of children with myocarditis diagnosed clinically and with biopsy confirmation (n=119) or with probable myocarditis diagnosed clinically or by biopsy alone (n=253) were compared with children with idiopathic dilated cardiomyopathy (n=1123). Characteristics at presentation were assessed as possible predictors of outcomes. The distributions of time to death, transplantation, and echocardiographic normalization in the biopsy-confirmed myocarditis and probable myocarditis groups did not differ (P≥0.5), but both groups differed significantly from the idiopathic dilated cardiomyopathy group (all P≤0.003). In children with myocarditis, lower LV fractional shortening z-score at presentation predicted greater mortality (hazard ratio, 0.85; 95% confidence interval, 0.73 to 0.98; P=0.03) and greater LV posterior wall thickness predicted transplantation (hazard ratio, 1.17; 95% confidence interval, 1.02 to 1.35; P=0.03). In those with decreased LV fractional shortening at presentation, independent predictors of echocardiographic normalization were presentation with an LV end-diastolic dimension z-score >2 (hazard ratio, 0.36; 95% confidence interval, 0.22 to 0.58; P<0.001) and greater septal wall thickness (hazard ratio, 1.16; 95% confidence interval, 1.01 to 1.34; P=0.04).

CONCLUSIONS

Children with biopsy-confirmed or probable myocarditis had similar proportions of death, transplantation, and echocardiographic normalization 3 years after presentation and better outcomes than those of children with idiopathic dilated cardiomyopathy. In children with myocarditis who had impaired LV ejection at presentation, rates of echocardiographic normalization were greater in those without LV dilation and in those with greater septal wall thickness at presentation. Clinical Trial Registration- URL: http://www.clinicaltrials.gov. Unique identifier: NCT00005391.

摘要

背景

心肌炎是儿童新发扩张型心肌病表型的一个病因,少数研究报告显示左心室(LV)功能恢复率较高。

方法和结果

比较了经临床和活检证实的心肌炎患儿(n=119)或经临床或单独活检诊断为可能心肌炎患儿(n=253)与特发性扩张型心肌病患儿(n=1123)的临床表现特征和结局。评估了临床表现的特点,作为结局的预测因素。在活检证实的心肌炎和可能心肌炎组中,死亡、移植和超声心动图正常化的时间分布没有差异(P≥0.5),但这两组与特发性扩张型心肌病组均有显著差异(均 P≤0.003)。在心肌炎患儿中,LV 短轴缩短率 Z 评分较低与更高的死亡率相关(风险比,0.85;95%置信区间,0.73 至 0.98;P=0.03),LV 后壁厚度较高与移植相关(风险比,1.17;95%置信区间,1.02 至 1.35;P=0.03)。在 LV 短轴缩短率降低的患儿中,超声心动图正常化的独立预测因素为 LV 舒张末期内径 Z 评分>2(风险比,0.36;95%置信区间,0.22 至 0.58;P<0.001)和室间隔厚度较大(风险比,1.16;95%置信区间,1.01 至 1.34;P=0.04)。

结论

经活检证实或可能的心肌炎患儿在出现症状后 3 年的死亡率、移植率和超声心动图正常化比例与特发性扩张型心肌病患儿相似,且预后更好。在出现 LV 射血分数降低的心肌炎患儿中,LV 无扩张和室间隔厚度较大的患儿超声心动图正常化率更高。临床试验注册- 网址:http://www.clinicaltrials.gov。独特标识符:NCT00005391。

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