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儿童肥厚型心肌病(hypertrophic cardiomyopathy,HCM)约占儿童心肌病的42%,每年病死率约为1%,成为一种严重危害儿童健康的疾病[1]。有研究认为,HCM属遗传性心脏病的一种,因其遗传基因和临床表型都存在很大的异质性,导致临床诊断儿童HCM面临许多困难,尤其是如何鉴别出高危HCM患儿更成为临床一大挑战[2-3]。心脏MRI拥有良好的空间和软组织分辨率,是目前诊断HCM最准确的方法,其Gd-DTPA心肌延迟强化显像被认为具有判断HCM预后的功能[4]。本研究收集了儿童HCM的心肌MRI资料,分析其临床相关影像学特征。
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71例HCM患儿中,纽约心功能分级(NYHA)Ⅰ级患儿15例、Ⅱ级34例、Ⅲ级19例、Ⅳ级3例;终末期HCM患儿3例,其中2例为非梗阻型对称性肥厚、1例为梗阻型非对称性肥厚;27例患儿具有明确HCM相关家族史,其中5例患儿有HCM相关心源性猝死家族史;超声心动图检查结果显示33例患儿为梗阻型,其中1例为左心室室中梗阻、2例为右心室梗阻;其他具体临床特征见表 1。X线胸片结果显示心胸比为45.4%±6.0%,3例患儿存在肺淤血。心电图检查结果显示64例患儿存在左心室高电压及ST-T改变。17例患儿接受心血管造影检查,其中4例患儿存在冠状动脉肌桥形成,并且均位于前降支。
项目 例数/(n,%) 性别(男/女) 46/25 晕厥 11(15.5) 胸痛不适 23(32.4) 收缩期杂音 41(57.7) 肥厚型心肌病家族史 27(38.0) 纽约心功能分级(Ⅰ/Ⅱ/Ⅲ/Ⅳ) 15/34/19/3 终末期肥厚型心肌病 3(4.3) 肥厚心肌类型 室间隔 60(84.5) 心尖 2(2.8) 向心性 9(12.7) 双心室受累 4(5.6) 超声心动图特征 梗阻型 33(46.5) 左心室室壁厚度≥30 mm 19(26.8) 药物治疗 β-阻滞剂 14(19.7) 钙离子拮抗剂 8(11.3) 利尿剂 2(2.8) 表 1 71例儿童肥厚型心肌病患儿临床特征
Table 1. Clinical characteristics of the children with hypertrophic cardiomyopathy
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MRI结果显示,4例(5.6%)患儿双心室受累(图 1),9例(12.7%)患儿左心室存在对称性肥厚,60例(84.5%)患儿存在室间隔肥厚(图 2),2例(2.8%)患儿心尖肥厚,3例(4.2%)患儿存在左心室心尖部室壁瘤形成。延迟强化成像结果显示,52例(73%)患儿存在心肌延迟强化,其中男性33例,女性19例,并且均位于肥厚心肌内,其中局限性强化占59%,弥漫性强化占41%。3例左心室心尖部室壁瘤形成患儿,室壁瘤存在透壁性强化。值得注意的是,2例恶性心律失常抢救成功的患儿和3例终末期HCM患儿左心室心肌均存在延迟强化。出现心肌延迟强化的患儿其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),但两组左心室室壁厚度[(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)。
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HCM患儿的平均随访时间为(2.4±1.6)年,心肌存在延迟强化的患儿有11例发生不良心血管事件,包括室颤2例(其中1例植入埋藏式心律转复除颤器),非持续性室速7例,充血性心力衰竭加重2例,心脏移植2例(病理学证实延迟强化心肌存在纤维瘢痕替代)。心肌无延迟强化的患儿组(图 3)随访期间未发生不良心血管病事件,与延迟强化的患儿组相比,差异有统计学意义(χ2=4.77,P=0.029)。
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
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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. -
表 1 71例儿童肥厚型心肌病患儿临床特征
Table 1. Clinical characteristics of the children with hypertrophic cardiomyopathy
项目 例数/(n,%) 性别(男/女) 46/25 晕厥 11(15.5) 胸痛不适 23(32.4) 收缩期杂音 41(57.7) 肥厚型心肌病家族史 27(38.0) 纽约心功能分级(Ⅰ/Ⅱ/Ⅲ/Ⅳ) 15/34/19/3 终末期肥厚型心肌病 3(4.3) 肥厚心肌类型 室间隔 60(84.5) 心尖 2(2.8) 向心性 9(12.7) 双心室受累 4(5.6) 超声心动图特征 梗阻型 33(46.5) 左心室室壁厚度≥30 mm 19(26.8) 药物治疗 β-阻滞剂 14(19.7) 钙离子拮抗剂 8(11.3) 利尿剂 2(2.8) -
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