原发性心内膜弹力纤维增生症患儿的超声心动图随访

Follow up Echocardiographic Analysis of Primary Endocardial Fibroelastosis Patients

目的：了解原发性心内膜弹力纤维增生症（简称心弹）患儿的心内结构及心功能改变的情况及其指导临床诊断、治疗及预后的价值。方法：心弹组随诊患儿２３例（均经地高辛治疗），年龄３～１７（７．２２±４．２０）岁。距初诊时间３年以上，设配对对照组年龄、性别、身长、体重一致的正常儿童２３名。应用Ｍ型超声心动图、二维超声心动图和多普勒彩色超声心动图测量心脏房室腔的大小、心室壁的厚度及运动，各瓣口血流频谱参数及心功能指数。结果：心弹患儿左心房、左心室明显缩小。除一例１４岁患儿心脏恢复正常外，其余仍轻度扩大。左心室收缩功能与对照组仍有差别。结论：心弹患儿经过３年以上的治疗病情明显好转，但左心收缩功能仍较对照组略低，左心亦较对照组大，应坚持长期治疗。

Objective:To get information about the changes of the heart chamber size and functions in follow up primary endocardial fibroelastosis (EFE) patients. Methods:Twenty three EFE patients (EFE group),aged 3 to 17 (7 22±4 2) years treated with digoxin and prednisone for more than 3 years and 23 normal children (control group) matched in age,sex,height and weight with those of the EFE group were studied.The heart chamber sizes,wall movements,quantities of blood flow and indexes of left and right ventricular systolic and diastolic function were measured with M mode,2 D and Doppler echocardiography. Results:The sizes of the left atrium and ventricle of the EFE patients were much smaller than the pretreatment levels.But the EFE group still had larger left atrial and ventricle sizes,lower EF and FS,longer left ventricle preejection time,and larger EPSS compared with the control group (all p <0 05). Conclusions:EFE patients have improved after three years treatment.The data also indicated that even though the EFE patients have significant clinical improvement,they still have poorer left ventricular function than the normal children and need long term treatment.