心内膜弹力纤维增生症患儿左室心肌重量指数、左心室收缩和舒张功能研究

A study on left ventricular mass index,systolic and diastolic function in children with endocardial fibroelastosis

目的：了解心内膜弹力纤维增生症（ＥＦＥ）患儿心室肥厚程度和左心室收缩舒张功能状态。方法：采用美国 Ａｃｕｓｏｎ１２８ＸＰ彩色多普勒超声仪检测１６例ＥＦＥ患儿左室心肌重量指数（ＬＶＭＩ）与心室收缩舒张功能，并与１７例相应年龄正常婴幼儿比较。结果工ＦＥ患儿 ＬＶＭＩ增加，左心室收缩功能减退。患儿左心室舒张功能减退表现为 Ｅ峰，ＡＣ，Ｅ／Ａ，ＥＦＦ及 １／３ＦＦ下降，ＩＶＲＴ延长，Ａ蜂和 ＡＦＦ增加。３例经治疗一年以上临床症状体征消失，心胸比率小于 ０． ５５。再次随访以上各项指标，显示ＥＦ，ＦＳ基本恢复正常，舒张功能部分改善，而３例ＬＶＭＩ虽明显下降，但仍均高于正常值。结论：ＥＦＥ患儿存在明显的心室肥厚，多普勒超声检测ＬＶＭＩ可安全无创准确了解ＥＦＥ患儿心肌肥厚程度。ＥＦＥ患儿不仅存在收缩功能异常且多合并以左心室松弛减退为主的舒张功能障碍。ＥＦＥ患儿临床恢复标准不仅依赖于临床症状体征及胸片好转．更需做左心功能和ＬＶＭＩ检测来综合评估。

Objective To search for the degree of ventricular hvpertrophy and the condition of ventricular systolic and diastolic function in children with endocardial fibroelastosis(EFE). Methods: The left ventricular mass index( LVMI) ,systolic and diastolic function were determined by Acuson 128XP color echocardiography instru ment made in the U. S. A. in 16 children with EFE and 17 age - matched normal children. Results : The LVMI was increased,and the left ventricular systolic function was decreased in the EFE cases. The reduction of the left ventricular diastolic function in children with EFE included the drop of E, AC, E/A, EFF and 1 /3FF, extension of LVRT, and increase of A and AFF. The indexes above were detected in 3 children with EFE whose clinical symptoms and signs had disappeared, whose cardiothoracic ratio ( CTR) was less than 0. 55 after treatment for more than 1 year. The results showed that the EF and FS basically recovered to normal levels. The diastolic function was only partly improved. The LVMIs of 3 cases were higher than those of the normal group though they were already obviously decreased. Conclusion: The ventricular hypertrophy was obvious in the children with EFE. The degree of ventricular hypertrophy was noninvasively clearly understcxxl by LVMI detected by color echocardiography. There was a reduction not only in systolic function but also in diastolic function mainly expressed by decrease of left ventricular relaxation in the children with EFE. The clinical recovery must be more fully evaluated not only by improvement in clinical symptoms, signs and chest X - ray study but also by detection of the cardiac function and LVMI.