心内膜弹力纤维增生症的超声诊断

目的:探讨心内膜弹力纤维增生症(endomyocardial fibroelastosis,EFE)的超声心动图诊断及鉴别诊断价值。方法:搜集EFE 15例,均行超声心动图检查,分析其超声表现及血流动力学变化。结果:二维超声示心内膜回声增强、增厚,厚度一般大于3.5mm,左房室内径增大11例,左右房室均增大3例,房室内径正常1例。二尖瓣轻度返流4例,中度3例,重度5例,无返流3例。11例治疗后房室腔缩小,左室功能(EF)改善,4例因病情较重死亡。结论:心内膜增厚、回声增强伴左房室腔扩大为EFE二维超声特征性表现,超声心动图对于EFE患儿的早期诊断、疗效评价具有重要的临床价值。

The diagnostic value of ultrasound in endomyocardial fibroelastosis

Objective:To investigate the echocardiographic value for diagnosis and differential diagnosis in endomyocardial fibroelastosis(EFE).Methods:15 cases with EHE who underwent echocardiography were retrospectively analyzed in the ultrasonographic appearances and hemodynamics.Results:Two-dimensional ultrasonogram demonstrated thicken and echogenic of endocardium,mean >3.5mm,with enlarged left atrioventricular cavity in 11 cases,left and right enlarged atrioventricular cavity in 3 cases,normal atrioventricular cavity in 1 case.There were 4 cases with mild mitral valve regurgitation(MVR),3 with midrange MVR,5 with severe MVR,3 with no MVR.The inner diameter of atrioventricular cavity contracted and systolic function of left ventricular improved in 11 cases,4 cases died of severe patient's condition.Conclusion:The thicken and echogenic endocardium accompanying with enlarged atrioventricular left cavity is characteristic ultrasonographic appearances of EFE,echocardiogram has important clinical value in early diagnosis and evaluation of therapeutic effect.