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肥厚型心肌病患儿的心血管磁共振特征。

Cardiovascular magnetic resonance characteristics in children with hypertrophic cardiomyopathy.

机构信息

Departments of Radiology, Pathology, and Nuclear Medicine, Cardiovascular Institute and Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

出版信息

Circ Heart Fail. 2013 Sep 1;6(5):1013-20. doi: 10.1161/CIRCHEARTFAILURE.113.000414. Epub 2013 Jul 19.

Abstract

BACKGROUND

Cardiovascular magnetic resonance has provided important information on the diagnosis and risk stratification of hypertrophic cardiomyopathy (HCM) in adults; however, comparable data are absent for HCM in children. The study was performed to evaluate cardiovascular magnetic resonance characteristics in children with idiopathic HCM.

METHODS AND RESULTS

Cardiovascular magnetic resonance was performed in 71 consecutive children with idiopathic HCM (age, 12.8±4.1 years; 46 boys and 25 girls). The left ventricular (LV) parameters indexed by body surface area were calculated and compared with those in 71 healthy children (age, 13.1±3.3 years; 51 boys and 20 girls). Among those with HCM, concentric LV hypertrophy occurred in 9 patients (12.7%): in 2 children, it progressed into end-stage phase. The prevalence of late gadolinium enhancement (LGE) was 73%, and the extent of LGE was 10.4±8.3% of LV mass. Children with HCM with LGE had greater LV mass indexed (112.7±57.9 versus 70.3±37.4 g/m(2); P=0.025) but had no significant difference in maximal LV wall thickness indexed (19.4±6.3 versus 18.1±7.9 mm/m(2); P=0.513). In addition, the hazard ratio of adverse events for the extent of LGE was 1.1 (P=0.004). The follow-up (2.4±1.6 years) demonstrated that LGE was associated with adverse events in children with HCM (log-rank; P=0.029).

CONCLUSIONS

The prevalence and extent of LGE in children with HCM approximate to those in adults with HCM. Furthermore, children with HCM with LGE tend to have adverse events, and larger researches with longer follow-up are required.

摘要

背景

心血管磁共振成像已为成人肥厚型心肌病(HCM)的诊断和危险分层提供了重要信息;然而,儿童 HCM 缺乏可比数据。本研究旨在评估特发性 HCM 患儿的心血管磁共振特征。

方法和结果

对 71 例连续的特发性 HCM 患儿(年龄 12.8±4.1 岁;46 名男孩和 25 名女孩)进行了心血管磁共振检查。根据体表面积计算左心室(LV)参数,并与 71 例健康儿童(年龄 13.1±3.3 岁;51 名男孩和 20 名女孩)进行比较。在 HCM 患儿中,9 例(12.7%)存在向心性 LV 肥厚:其中 2 例进展为终末期。延迟钆增强(LGE)的发生率为 73%,LGE 的范围为 LV 质量的 10.4±8.3%。有 LGE 的 HCM 患儿的 LV 质量指数(112.7±57.9 比 70.3±37.4 g/m²;P=0.025)更大,但 LV 最大壁厚度指数(19.4±6.3 比 18.1±7.9 mm/m²;P=0.513)无显著差异。此外,LGE 范围的不良事件风险比为 1.1(P=0.004)。2.4±1.6 年的随访显示,LGE 与 HCM 患儿的不良事件相关(对数秩检验;P=0.029)。

结论

HCM 患儿的 LGE 发生率和范围与 HCM 成人相似。此外,有 LGE 的 HCM 患儿易发生不良事件,需要进行更大规模、随访时间更长的研究。

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