儿童肥厚型心肌病磁共振延迟强化特征分析

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。

Characteristics of late gadolinium enhancement in children with hypertrophic cardiomyopathy

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.