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中华妇幼临床医学杂志(电子版) ›› 2018, Vol. 14 ›› Issue (01) : 57 -61. doi: 10.3877/cma.j.issn.1673-5250.2018.01.009

所属专题： 文献；

论著

儿童心肌致密化不全的临床与超声心动图特点
刘丹1, 刘瀚旻2, 谢亮3, 郭楠1, 伍婷1, 余莉4, 陈娇1,†()   
  1. 1. 610041　成都，四川大学华西第二医院超声科、出生缺陷与相关妇儿疾病教育部重点实验室
    2. 610041　成都，四川大学华西第二医院儿童呼吸科
    3. 610041　成都，四川大学华西第二医院西部妇幼医学研究院血管重构与发育缺陷研究室
    4. 610041　成都，四川大学华西第二医院儿童心血管科
  • 收稿日期:2017-10-27 修回日期:2018-01-16 出版日期:2018-02-01
  • 通信作者: 陈娇

Clinical features and characteristics of echocardiography of noncompaction of the ventricular myocardium in children

Dan Liu1, Hanmin Liu2, Liang Xie3, Nan Guo1, Ting Wu1, Li Yu4, Jiao Chen1,†()   

  1. 1. Department of Ultrasound, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education
    2. Department of Pediatric Respiratory, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education
    3. Vascular Remodeling and Developmental Defects Research Unit, West China Institutes for Women and Children′s Health, Chengdu 610041, Sichuan Province, China
    4. Department of Pediatric Cardiology, West China Second University Hospital, Sichuan Universtity, Chengdu 610041, Sichuan Province, China
  • Received:2017-10-27 Revised:2018-01-16 Published:2018-02-01
  • Corresponding author: Jiao Chen
  • About author:
    Corresponding author: Chen Jiao, Email:
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引用本文：

刘丹, 刘瀚旻, 谢亮, 郭楠, 伍婷, 余莉, 陈娇. 儿童心肌致密化不全的临床与超声心动图特点[J/OL]. 中华妇幼临床医学杂志(电子版), 2018, 14(01): 57-61.

Dan Liu, Hanmin Liu, Liang Xie, Nan Guo, Ting Wu, Li Yu, Jiao Chen. Clinical features and characteristics of echocardiography of noncompaction of the ventricular myocardium in children[J/OL]. Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition), 2018, 14(01): 57-61.

目的

探讨儿童心肌致密化不全(NVM)的临床及超声心动图特点。

方法

回顾性分析2013年8月至2017年8月，于四川大学华西第二医院经超声心动图诊断为NVM的74例患儿病例资料，总结儿童NVM临床特点与超声心动图特点。

结果

①本组74例儿童NVM的主要门诊/入院诊断为：先天性心脏病占32.4%(24/74)，NVM占28.4%(21/74)，肺炎占21.6%(16/74)。主要临床表现为：心前区收缩期杂音(31.1%，23/74)，心力衰竭(25.7%，19/74)，心影增大(17.6%，13/74)及心律失常(4.1%，3/74)等。②本组74例儿童NVM的超声心动图特点为：91.9%(68/74)为左室型NVM(LVNC)；左心室受累为95.9%(71/74)，以心尖合并心尖部下壁及侧壁受累、心尖受累为主。超声心动图测量致密心肌厚度为(2.28±0.86) mm，非致密心肌厚度为(6.94±1.75) mm，非致密心肌厚度与致密心肌厚度比值为(3.50±1.76)。本组大部分病例心腔增大，并且以左房、室为主；左心室收缩功能减低为33.8%(25/74)。孤立性NVM为60.8%(45/74)，非孤立性NVM主要合并先天性心脏病(25.7%，19/74)及扩张性心肌病(8.1%，6/74)。74例患儿均无心内血栓形成。

结论

儿童NVM的临床表现无特异性，临床诊断较困难。超声心动图能观察心肌形态、评价心肌运动及诊断心内合并畸形，是确诊儿童NVM的首选影像学检查方法。

关键词: 心肌疾病, 超声心动描记术，多普勒，彩色, 儿童
Objective

To investigate the clinical and echocardiographic characteristics of children with noncompaction of the ventricular myocardium (NVM).

Methods

A total of 74 case informations of NVM children who were definitely diagnosed by echocardiography in West China Second University Hospital, Sichuan University from August 2013 to August 2017, were retrospectively reviewed, and clinical and echocardiographic characteristics of NVM in children were summarized.

Results

①Clinical characteristics in 74 cases of NVM children: the main outpatient or admitting diagnosis were congenital heart disease accounting for 32.4% (24/74), NVM accounting for 28.4%(21/74), pneumonia accounting for 21.6%(16/74). The main manifestations were precardiac systolic murmur (31.1%, 23/74), heart failure (25.7%, 19/74), heart shadow enlargement (17.6%, 13/74) and arrhythmia (4.1%, 3/74), etc.. ② Echocardiographic characteristics in 74 cases of NVM children: left ventricular noncompaction (LVNC) was 91.9% (68/74); left ventricle involvement was 95.9% (71/74), the main abnormal segments were cardiac apex combined with inferior and lateral walls of cardiac apex, and cardiac apex. Compacted layer of myocardium was (2.28±0.86) mm and noncompacted layer was (6.94±1.75) mm measured by ultrasoundcardiogram, and the ratio of noncompacted to compacted layers was (3.50±1.76). Most of cases were chambers of heart enlarged, and mainly in left atrium and ventricle. And 33.8% (25/74) of NVM children were left ventricular systolic function decreased. Isolated NVM was 60.8% (45/74), non-isolated NVM mainly complicated with congenital heart diseases (25.7%, 19/74) and dilated cardiomyopathy (8.1%, 6/74). There was no thrombosis in all cases.

Conclusions

The children with NVM have no specific clinical manifestations, it is difficult to make clinical diagnosis of NVM in children. Echocardiography is the preferred imaging examination method to make a definite diagnosis of NVM in children for its observation of myocardial morphology, evaluation of myocardial motion, and diagnosis of intracardiac combined malformations.

Key words: Cardiomyopathies, Echocardiography, doppler, color, Child
图1 1例女性心肌致密化不全患儿(9岁)的超声心动图声像图(图1A为二维超声心动图声像图，显示左心室心肌呈双层改变，下层致密心肌变薄，内层非致密心肌明显增厚、回声增强，形成多个深陷隐窝，见黄色箭头；图1B为彩色多普勒血流成像声像图，显示左心室粗大肌小梁，隐窝内血流与心腔相通，见黄色箭头)
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