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 ～18 kg,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)0.26～0.48(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)复查EF有所改善[0.38～0.55(0.45±0.08),P＜0.05],部分增厚的心内膜消失或变薄(3例),部分仍存在(2例).结论 对冠状动脉起源异常的继发性心内膜弹力纤维增生症早期明确诊断,早期矫治解剖畸形,可完全逆转心内膜弹力纤维增生.对于合并主动脉缩窄和主动脉瓣狭窄等左心系统畸形的继发性心内膜弹力纤维增生症,外科手术治疗有助于心功能的恢复,但是否可逆转心内膜弹力纤维增生仍有待进一步的随访.对于严重心功能不全患儿心脏移植可能是最佳治疗方案.%Objective To investigate surgical treatment and effect of secondary endocardial fibroelastosis,based on respective analysis of clinical data and follow-up data of patients with secondary endocardial fibroelastosis (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 ± 0.08 (0.26 ～ 0.48)].All patients except 2 patients with anomalous origin of the coronary artery received treatment of digitaloid drugs before operation,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 complicated with anomalous origin of the left coronary artery from the pulmonary artery,who were underwent correction of anomalous origin 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 postoperative pathological examination showed obviously positive dyeing of elastic fibers.In 3,6,12 and 24 months after operation,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 obtained post-operative good recovery,and the Untrasonic Cardiogram show disappearance of endocardial fibroelastosis.The post-operative mean time of using respirator(4.0 ± 1.5) days (2-7 days).Compared with the preoperative data,the cardiac function index (EF) was not significantly better at 2 weeks and 3-6 months[0.38 ± 0.07 (0.28 ～ 0.48),P ＞ 0.05 ; 0.39 ± 0.08 (0.30 ～ 0.50),P ＞ 0.05],and the non-resected fibroelatic endocardium still existed and were not attenuated.But the cardiac function index (EF) significantly increased [0.44 ± 0.08 (0.38 ～ 0.55),P ＜ 0.05] than the pre-operative EF,and the 3 of 5 cases the fibroelatic endocardium were attenuated or disappeared,while 2 of 5 cases the fibroelatic endocardium still existed.Conclusion SEF is the important causes of the infant intractable heart failure,which has the characteristic of high mortality and limited therapy.For SEF patients with anomalous origin of the coronary artery,the SEF is completely reversed by early diagnosis and early correction of the malformation.For SEF patients with CoA or aortic stenosis,the surgical treatment could promote recovery of cardiac function,but whether the SEF were reversed is still subject to further follow-up.The heart transplantation is the best therapy for SEF with severse heart failure.
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