儿童肥厚型心肌病磁共振延迟强化特征分析

Characteristics of late gadolinium enhancement in children with hypertrophic cardiomyopathy

    摘要
  • 摘要:
    目的分析肥厚型心肌病（HCM）患儿心脏受累心肌MRI对比剂延迟强化发生率及其与预后的相关性。
    方法收集2006年1月至2012年1月我院收治的HCM患儿71例，所有患儿均接受心脏MRI检查，并行对比剂增强心肌显像。根据左心室心肌是否存在延迟强化，将患儿分为延迟强化组和非延迟强化组，并进一步分析受累心肌对比剂延迟强化的范围及程度等。应用Kaplan-Meier生存曲线分析两组患儿的预后差别，两组临床数据间的统计分析采用t检验。
    结果71例HCM患儿中，9例（12.7%）患儿左心室为对称性肥厚，其中2例患儿进展至终末期。52例（73%）患儿出现心肌延迟强化，其延迟强化组患儿左心室质量高于非延迟强化组患儿（112.7±57.9）g/m2 vs.（70.3±37.4）g/m2，两组间差异有统计学意义（t=2.71，P=0.025）；但两组左室室壁厚度（19.4±6.3）mm/m2 vs.（18.1±7.9）mm/m2比较，差异无统计学意义（t=0.69，P=0.513）。HCM患儿随访（2.4±1.6）年显示，心肌延迟强化与不良心血管事件的发生密切相关（χ2=4.77，P=0.029）。
    结论HCM患儿中，其心肌延迟强化发生率与成人患者接近，并且心肌延迟强化具有判断患儿预后的临床价值。

     

    Abstract:
    ObjectiveTo analyze the prevalence of late gadolinium enhancement (LGE) in children with idiopathic hypertrophic cardiomyopathy(HCM).
    MethodsCardiac magnetic resonance imaging(CMR) was performed consecutively in 71 children with idiopathic HCM(12.8 ±4.1 years old; 46 males, 25 females) from January 2006 to January 2012. Left ventricular (LV) parameters indexed by the body surface area were calculated, and LGE was carried out. Kaplan-Meier survival curves were generated, and differences in the two groups were compared via a log-rank test.
    ResultsOf the total number of children with HCM, 9 patients (12.7%) had concentric LV hypertrophy, and 2 of these 9 patients progressed into an end-stage phase. The prevalence of LGE was 73%. The LV mass index of the children with HCM subjected to LGE was greater than that of the other group(112.7±57.9) g/m2 vs. (70.3±37.4) g/m2, t=2.71, P=0.025, but their maximal LV wall thickness index was not significantly different(19.4±6.3) vs. (18.1±7.9) mm/m2, t=0.69, P=0.513. Follow-up (2.4±1.6 years) revealed that LGE was associated with the adverse events in children with HCM (log-rank, χ2=4.77, P=0.029).
    ConclusionsThe prevalence of LGE in childhood HCM was similar to that in adult HCM. Children with HCM subjected to LGE likely suffered from adverse events.

     

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