心室肌致密化不全的超声心动图诊断

Diagnosis of noncompaction of the ventricular myocardium by echocardiography

目的采用超声心动图对心室肌致密化不全（NCVM）进行诊断,阐明其超声心动图特征。方法将2006年5月至2015年5月接受超声心动图检查、且诊断为NCVM的患儿53例纳入研究,其中男28例,女25例,首次诊断年龄为15 d至18岁。采用经胸二维超声心动图和彩色多普勒进行定性诊断,于胸骨旁心室短轴切面观测量舒张末期疏松部/致密部（N/C）比值作为定量诊断标准。结果在53例患儿中,均观测到过度突出的肌小梁和深陷的小梁间隐窝,且心腔内的血流与肌小梁间的隐窝沟通。NCVM累及的部位主要是心尖部（100%）和左室侧壁中间段（98%）,其次为左室后壁中间段（49%）和左室下壁中间段（42%）。53例NCVM患儿中,N/C比值为4.3±1.9（2.1～10.0）≥2。心功能不全占83%（44/53）,这些患儿的左室射血分数为43%±9%。结论超声心动图可以对NCVM进行定性和定量诊断,并对其心功能进行评价。NCVM以累及心尖部和左室侧壁中间段较常见,且常伴有左室射血分数减低。

Objective To evaluate the value of echocardiography in the diagnosis of noncompaction of the ventricular myocardium（NCVM） and to elucidate the echocardiographic characteristics of NCVM. Methods This study included 53 patients（28 boys and 25 girls）, with an age for initial diagnosis of 15 days to 18 years, who were diagnosed with NCVM by echocardiography between May 2006 and May 2015. Transthoracic two-dimensional echocardiography and color Doppler were performed for qualitative diagnosis, and the end-diastolic non-compacted layer/compacted layer（N/C） ratio measured in the parasternal ventricular short-axis sectional view was selected as the criterion for quantitative diagnosis. Results The excessively prominent ventricular trabeculae and deep inter-trabecular recesses were all seen in 53 cases, and the blood flow in the cardiac chambers was connected to the inter-trabecular recesses. The areas involved in NCVM were mainly the apex（100%） and the middle segment of the left ventricular lateral wall（98%）, followed by the middle segment of the left ventricular posterior wall（49%） and the middle segment of the left ventricular inferior wall（42%）. In 53 children with NCVM, the N/C ratio was 4.3±1.9（2.1-10.0）. Cardiac insufficiency was found in 83%（44/53） of the children with NCVM, and the left ventricular ejection fraction for these children was（43±9）%. Conclusions Echocardiography can be used in the qualitative and quantitative diagnosis of NCVM and in the evaluation of cardiac function. The apex and the middle segment of the left ventricular lateral wall are often involved in NCVM, accompanied by decrease in the left ventricular ejection fraction.