· 临床研究·

    作者单位:100029 北京,首都医科大学附属北京安贞医院超声诊

    断科(李治安、何怡华、栾姝蓉) ,病理科(张惠信)

    心肌致密化不全的超声心动图与病理检查

    对照研究

    何怡华 李治安 张惠信 栾姝蓉

    【摘要】 目的 探讨心肌致密化不全(NVM)的超声心动图表现与病理特征 ,以期尽早诊断并预防致

    死性心律失常和栓塞发生。方法 超声心动图检查方法以四腔观、左心室长轴二腔观和左心室短轴观为

    主进行多切面扫查 ,重点观察近心尖1/ 3的左心室心肌与心内膜。结果 24 例患者超声心动图检查均可

    见受累心腔内异常隆突、粗大的肌小梁和其间深陷的隐窝影像 ,其中 3 例合并其他先天性心脏畸形 ,20 例

    伴有充血性心力衰竭症状 ,仅2例无任何症状。24例中13例行冠状动脉造影未见异常 ,2例因严重充血性

    心力衰竭而最终进行心脏移植。对受体心脏进行了病理解剖和组织学检查 ,受累心腔内显示有异常粗大

    的肌小梁和交错深陷的隐窝 ,从心底到心尖致密心肌逐渐异常变薄;心内膜为增厚的纤维组织 ,内层非致

    密心肌肌束粗大紊乱、细胞核异形 ,外层致密心肌肌束走行及形态基本正常 ,细胞核大小均匀。结论 心

    肌致密化不全具有特征性超声心动图改变和特有的病理组织学变化 ,可区别于原发性扩张型心肌病。超

    声心动图是诊断心肌致密化不全的重要手段。

    【关键词】 超声心动描记术;心脏病;心肌疾病

    Comparative study between echocardiographic manife stations and pathological examinations in noncompaction

    of ventricular myocardium HE Yi2hua , LI Zhi2an , ZHANG Hui2xin , et al . Department of Echocardiography ,Beijing Anzhen Hospital , Capital University of Medical Sciences , Beijing 100029 ,China

    【Abstract】 Objective By exploring echocardiographic characters and pathologic examination of noncompacted

    ventricular myocardium (NVM) , to prevent life2threatening arrhythmias and embolism. Methods Echocardiographic

    examinations included four 2chamber view , two2chamber view and short axis view of left ventricle , with an emphasis on

    observing left ventricular myocardium and endocardium approach to one 2third of apex of heart . Re sults All 24 patients

    showed obvious echocardiographic findings characterized by prominent and excessive myocardial trabeculations and deep

    intratrabecular recesses in the affected ventricular cavity. Three cases complicated with other congenital heart

    malformations , 20 cases with congestive heart failure and only 2 cases were asymptomatic. Coronary arteriography was

    performed in 13 cases and revealed normal findings. Two cases eventually underwent heart transplantation because of

    severe congestive heart failure. Gross and histological findings demonstrated prominent muscular trabeculations , with deep

    intratrabecular recesses into lesion heart . Serial section from the base of the ventricle toward the apex revealed gradually

    weaker myocardium. Noncompaction of ventricular myocardium showed a thin , compacted epicardial and an extremely

    thickened endocardium by fibrous tissue. Conclusions Noncompaction of ventricular myocardium has characteristic

    echocardiographic manifestations and specific pathologic changes , which are different from those of primary enlarged type

    of cardiomyopathy.

    【Key words】 Echocardiography ;Heart diseases ;Myocardial diseases

    心肌致密化不全 ( noncompaction of ventricular

    myocardium , NVM)为一种罕见的先天性疾病 ,有家族

    发病倾向。心肌致密化不全可单独存在 ,称孤立性心

    肌致密化不全 ( isolated noncompaction of ventricular

    myocardium, INVM) ,或与其他先天性心脏畸形并存。

    对该病的诊断多以超声心动图为依据。本研究总结了

    我院近年来发现的 24 例 NVM的特征性超声改变 ,并

    对实施心脏移植的2例受体心脏进行了病理解剖和组

    织学观察。

    资 料 与 方 法

    一、研究对象

    自2000年8月至2002年12月以来在我院门诊就

    诊或住院患者24例 ,男 18 例 ,女 6 例 ,年龄 2～71 岁 ,平均(36 ±25)岁。所有患者均进行临床体检、超声心

    动图、十二导联心电图检查 ,部分患者行磁共振、 201

    铊

    心肌显像、冠状动脉造影等检查。

    · 185 · 中华超声影像学杂志2003年10月第12卷第10期 Chin J Ultrasonogr ,October 2003 ,Vol 12 ,No. 10

    ? 1995-2005 Tsinghua Tongfang Optical Disc Co., Ltd. All rights reserved.二、仪器与方法

    应用 Acuson Sequoia 512、 HP Sonos 5500、 HP Sonos

    4500彩色多普勒超声诊断仪。探头频率为 2～4 MHz。

    超声心动图主要观察左心室长轴观和短轴观、胸骨旁

    或心尖四腔观、剑突下四腔观。所有资料均采用磁光

    盘存储 ,统一脱机分析 ......