小儿心内膜弹力纤维增生症76例临床分析

Clinical analysis of 76 cases with endocardial fibroelastosis

回顾性分析1998年6月至2009年12月住院的76例心内膜弹力纤维增生症（EFE）患儿的临床资料以及随访结果.结果示,EFE临床表现以呼吸急促、咳嗽、发绀为主要症状,均有不同程度心力衰竭.心电图检查以窦性心动过速、左室肥厚、ST-T波改变为主.X线检查心胸比例＞0.65者54例（71%）;肺炎33例（43%）.超声心动图主要表现为左室扩大、搏动减弱,其中左室收缩功能降低76例（100%）,左室舒张功能降低21例（28%）,左室心内膜增厚、回声增强63例（83%）,室间隔增厚11例（14%）.患儿入院后经洋地黄等治疗,好转出院58例（76%）,自动出院10例（13%）,死亡8例（11%）.好转出院的58例中有41例接受了定期随访,随访时间1～4年,平均2.6年,结果68%（28/41）患儿临床治愈,17%（7/41）病情恶化,15%（6/41）因未坚持用药而死于心力衰竭.提示超声心动图能检测EFE的特征性表现,监测心脏收缩功能和舒张功能;肺部感染可能是促使病情恶化的重要因素之一;坚持治疗为决定预后的主要因素之一.

The clinical data of 76 children with EFE admitted from June, 1998 to December, 2009 was retrospectively analyzed. Tachypnea、 cough and cyanosis were common and all presented with heart failure. Electrocardiogram revealed sinus tachycardia 、left ventricular hyperthrophy and ST-T alterations. On chest radiograph 54 cases （54/76） had a cardiothoracic ratio 〉 0. 65 and 33 with pneumonia.Echocardiopraphy revealed lowered left ventricular systolic function in all cases and decreased left ventricular diastolic function in 21 （21/76）. 63 cases （63/76） presented with thickened and enhanced left ventricular endocardium and 11 （11/76） with thickened interventricular septals. All were treated with digitalis. 58children were improved and discharged, 10 gave up therapy and 8 died during hospitalization. 41 out of 58 improved cases were followed for 1 - 4 years, with 2. 6 years on average. Among them 28 were clinically cured, 7 deteriorated and 6 died of heart failure. Echocardiography is of diagnostic value for ERE. Systolic and diastolic function of the heart can be used to predict the outcome. Pulmonary infection is a major risk factor associated with deterioration. Long-term treatment is imperative to improve the prognosis.