小儿心内膜弹力纤维增生症60例临床特点分析及结局

目的：分析心内膜弹力纤维增生症（ EFE）的临床表现特点和实验室、影像学检查特点，并随访患儿的治疗结果。方法回顾性分析2000年12月至2011年6月在湖北十堰市太和医院儿2科住院的60例小儿EFE患者，对其临床症状和体征、实验室和影像学检查结果进行总结，并统计患儿的随访结果。结果 EFE患儿以肺炎、心衰为主要首诊原因（55．0％）；临床症状主要有咳嗽（75．0％）、呼吸困难（68．3％）、气促（83．3％）。心电图显示的主要异常为：左室肥大伴劳损（38．3％），单纯左室肥大（15．0％），窦性心动过速（30．0％），ST-T改变（11．7％）。胸部X线检查心脏普遍增大（100．0％），心胸比例＞0．60者占38．3％。超声心动图显示的主要异常有：左心室均扩大（100．0％），左室心内膜增厚、回声增强（81．7％），左室收缩功能均降低（100％），左室射血分数（EF）均低于55％（100％）。经治疗，临床好转41例（68．3％），自动出院12例（20．0％），死亡7例（11．7％）。对53例出院患儿进行随访，6例失访，47例患儿中4例（8．5％）病情加重，1例（2．1％）死于心力衰竭。结论超声心动图能检测EFE的特征性表现，坚持治疗可以改善患儿预后。

Clinical features and outcomes of endocardial fibroelastosis in 60 children

Objective To analyze the clinical features, laboratory and imaging characteristics of endocardial fibroelastosis （ EFE） in children, and to follow up treatment outcomes.Methods Retrospective study was conducted on 60 cases with EFE admitted in the second department of pediatrics in Taihe Hospital of Shiyan during the period of December 2000 to June 2011.Their clinical features, physical signs, laboratory and imaging characteristics were summarized and follow-up results were recorded.Results The main causes of the first visit of EFE children were pneumonia and heart failure （55%）.Cough （75.0%）, dyspnea （68.3%） and shortness of breath （83.3%） were major clinical symptoms.The main changes in electrocardiogram included left ventricular hypertrophy with strain （38.3%）, left ventricular hypertrophy alone （15.0%）, sinus tachycardia （30.0%） and ST-T changes （11.7%）.Chest X-ray examination showed larger heart （100.0%） and cardiothoracic ratio〉0.60 （38.3%）.Echocardiography showed expanded left ventricular （100.00%）, thickened left ventricular endocardium, echo enhancement （ 81.7%） , decreased left ventricular systolic function （ 100%） , and left ventricular ejection fraction （EF） less than 55%（100%）.After treatment, 41 cases were improved （68.3%）, 12 cases were discharged （20.0%） and 7 cases died （11.7%）.Six cases were lost in follow-up.Of 47 cases with follow-up the disease aggravated in 4 cases （8.5%） and 1 case （2.1%） died of heart failure.Conclusion Echocardiography can detect the characteristic performance of EFE and long-term treatment can improve the prognosis of patients.