小儿心内膜弹力纤维增生症59例诊断及随访临床分析  

Study of endocardial fibroelastosis of 59 child cases by echocardiography on diagnosis and follow-up

作　　者：南晓琴[1] 任杰[1] 魏秀勤[1] 

机构地区：[1]山西省儿童医院

出　　处：《山西医药杂志》2003年第3期208-210,共3页Shanxi Medical Journal

摘　　要：目的　探讨超声心动图在小儿心内膜弹力纤维增生症 ( EFE)诊断随访中的价值及影响 EFE预后的相关因素。方法　对 5 9例 EFE患儿起病时临床表现、心功能状态及超声学指标进行了回顾性分析。随访 3 4例 ,时间 1～ 18a,平均 ( 7± 5 ) a。结果　经超声心动图检查所有患儿均得以早期诊断。起病年龄 2个月～ 10岁 ,其中 1岁内发病 46例 ( 78% )。主要超声改变 :左室扩大、室壁及室间隔活动僵硬 5 9例 ( 10 0 % ) ;左室收缩功能明显下降 5 4例 ( 92 % ) ;内膜增厚 ,回声增强 47例 ( 80 % )。 3 4例随访结果 :临床治愈 15例 ,好转 13例 ,持续 /反复心功能不全 5例 ,死亡 1例。临床治愈病人 ,超声检查仍可见心脏结构和组织学异常。结论　超声心动图在 EFE诊断及随访中具有重要的临床应用价值。临床治愈病人其心脏结构和组织学改变是否可以完全恢复正常尚需进一步随访观察。Objective Evaluation of echocardiography for diagnosis and follow-up of endocardial fibroelastosis (EFE) and of prognostic factors.Methods A retrospective clinical study of 59 cases with EFE was carried out based on the data from the patients′ records.Clinical and echocardiographic findings at presentation were analyzed.Long-term follow-up data were available in 34 cases.The average follow-up was 7±5 years (range,1 year to 18 years).Results By echocardiographic examination,all of the cases were early diagnosed.Of the 59 cases aged from 2 months to 10 years,46 cases (78%) were under one year.Prominent echocardiographic features included dilatation of left ventricule with poor movement and contraction of left ventricular posterior wall and septa (100%),significantly reduced contractility (92%) and the thickening and echo strengthening of endocardium (80%).Among the 34 followed-up cases,15 cases clinically cured,13 cases in good evolution,5 cases survived with persistent left ventricular dysfunction and 1 case died.Even in those clinically cured cases,there still had been residual cardiac structural and histological lesions as judged by the endocardiogram.Conclusion With characteristic manifestation for EFE and its advantages of non-invasion,safty and easy-operating,echocardiography is of great value for early diagnosis and follow-up of EFE.It needs a long-term follow-up to decide if cardiac structure and histological changes can be completely recovered.Patients with persistent symptoms of poor cardiac function or congestive heart failure and those with recurrent symptoms after primary therapy and long-term treatment tend to have a poor outcome.

关 键 词：心内膜弹力纤维增生症 诊断 随访 临床分析 儿童 超声心动图 治疗 

分 类 号：R725.4[医药卫生—儿科]