CAJ | 학술논문

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.
회고성분석1998년6월지2009년12월주원적76례심내막탄력섬유증생증(EFE)환인적림상자료이급수방결과.결과시,EFE림상표현이호흡급촉、해수、발감위주요증상,균유불동정도심력쇠갈.심전도검사이두성심동과속、좌실비후、ST-T파개변위주.X선검사심흉비례＞0.65자54례(71%);폐염33례(43%).초성심동도주요표현위좌실확대、박동감약,기중좌실수축공능강저76례(100%),좌실서장공능강저21례(28%),좌실심내막증후、회성증강63례(83%),실간격증후11례(14%).환인입원후경양지황등치료,호전출원58례(76%),자동출원10례(13%),사망8례(11%).호전출원적58례중유41례접수료정기수방,수방시간1～4년,평균2.6년,결과68%(28/41)환인림상치유,17%(7/41)병정악화,15%(6/41)인미견지용약이사우심력쇠갈.제시초성심동도능검측EFE적특정성표현,감측심장수축공능화서장공능;폐부감염가능시촉사병정악화적중요인소지일;견지치료위결정예후적주요인소지일.
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.