继发性心内膜弹力纤维增生症的外科治疗  

Surgical treatment of secondary endocardial fibroelastosis

作　　者：王国华[1] 苏伟[1] 王思桦[1] 程超[1] 童玲[2] 夏家红[1] 董念国[1] 

机构地区：[1]华中科技大学同济医学院附属协和医院心血管外科,430022 [2]华中科技大学同济医学院附属协和医院病理科,430022

出　　处：《中华胸心血管外科杂志》2013年第11期656-659,共4页Chinese Journal of Thoracic and Cardiovascular Surgery

基　　金：国家自然科学基金青年科学基金项目（81102240）.

摘　　要：目的探讨继发性心内膜弹力纤维增生症（endocardial fibroelastosis，EFE）的外科治疗方法及效果。方法2010年1月至2012年12月共收治继发性心内膜弹力纤维增生症患儿10例，男7例，女3例。年龄1个月-4岁，平均（9．7±13．7）个月。体质量4～18kg，平均（9．0±3．9）kg。术前均通过超声心动图和／或心脏大血管CTA检查明确诊断，均存在不同程度的心功能不全，左心室射血分数（EF）O．26—0．48（0．37±0．08）。除2例合并冠状动脉起源异常外，术前均行洋地黄类药物治疗，改善术前心功能。5例合并降主动脉缩窄（其中1例伴二尖瓣关闭不全和动脉导管未闭），其中2例行降主动脉缩窄矫治，2例行降主动脉缩窄矫治和部分心内膜切除术，1例行降主动脉矫治、二尖瓣整形和部分心内膜切除术；2例合并主动脉瓣狭窄，行主动脉瓣交界切开和部分心内膜切除术；2例合并冠状动脉起源异常，行冠脉起源异常矫治术；1例合并二尖瓣狭窄并关闭不全，行二尖瓣置换术。术中肉眼见弹性纤维增生的心室内膜呈不透明的灰白色，心内膜切除患儿术后病理检查示弹力纤维染色阳性，并于术后3、6、12、24个月随访，超声心动图评估心功能、心内膜改变。结果术后死亡1例，为6月龄左冠状动脉起源于肺动脉患儿，死于术后严重低心排血量综合征。而1月龄左冠状动脉起源于肺动脉的患儿术后恢复良好，2年后复查超声心动图提示心内膜弹力纤维增生完全消失。术后脱离呼吸机时间2—7天，平均（4．0±1．5）天。术后2周复查超声心动图均显示术后EF较术前无明显改善[0．28～0．48（0．38±0．07），P〉0．05]，未切除增厚的心内膜均存在。术后3～6个月复查超声心动图示EF亦无显著改善[0．30～0．50（0．39±0．08），P〉0．05]，未切除增厚的心内膜无明显改变。术后1～2年（5例）�Objective To investigate surgical treatment and effect of secondary endocardial fibroelastosis, based on re- spective analysis of clinical data and follow-up data of patients with secondary endocardial fibreelastosis （SEF） between 2010 and 2012. Methods A retrospective analysis was performed including 10 patients with secondary endocardial fibroelastosis from January 2010 to December 2012 in Wuhan Union Hospital. All patients were diagnosed by Untrasonic Cardiogram and/or CT angiography of heart and great vessel, and had cardiac insufficiency in different degree [ EF 0.37± O. 08 （0.26 ± 0.48 ） ]. All patients except 2 patients with anomalous origin of the coronary artery received treatment of digitaloid drugs before opera- tion, which promoted preoperative cardiac function. 5 patients with SEF complicated with Congenital Coarctation of the Aorta （ CoA）, 2 patients underwent correction of CoA, 2 patients underwent correction of CoA and partial resection of endocardium, 1 patient underwent correction of CoA, partial resection of endocardium and mitral vavuloplasty. 2 patients with SEF complicat-ed with anomalous origin of the left coronary artery from the pulmonary artery, who were underwent correction of anomalous ori- gin of coronary artery. 2 patients with SEF complicated with aortic stenosis, who were underwent aortic commissurotomy and partial resection of endocardium. 1 patient with SEF complicated with mitral stenosis and insufficiency, who underwent mitral valve replacement. The intraopertive gross appearance of endocardium was opaque greyish-white not transparent pink. The post- operative pathological examination showed obviously positive dyeing of elastic fibers. In 3, 6, 12 and 24 months after opera- tion, Untrasonic Cardiogram evaluated cardiac function and endocardium. Results one 6 months patients with origin of left coronary from pulmonary artery died of severe post-operative low cardiac output syndrome, while another 1 months patients with origin of left coronary from pulmonary artery

关 键 词：心内膜弹力纤维增生症 继发性 外科治疗 

分 类 号：R654.2[医药卫生—外科学]