摘要
目的总结小儿心肌致密化不全(NVM)的临床特征,以提高对该病的认识及诊治水平。方法回顾性分析2005年1月-2010年12月在本院住院的25例NVM患儿的临床表现、心脏彩超、ECG等辅助检查结果及治疗、随访情况。结果 25例患儿中有心功能不全表现者19例(76%);有心律失常者7例(28%),其中室性快速心律失常4例(16%);均未发现心内膜血栓形成。25例患儿彩色多普勒超声心动图均见典型的NVM改变,左心室受累23例(92%),右心室受累2例(8%);心脏扩大24例(96%),左心室射血分数<50%20例(80%)。住院前误诊为扩张型心肌病(DCM)3例,心内膜弹力纤维增生症(EFE)1例。随访0.4～5.6 a,6例患儿死于顽固性心力衰竭,1例患儿猝死,4例失访。结论小儿NVM的临床表现多以心功能不全及室性快速心律失常为主,极少形成心内膜血栓。小儿NVM易误诊为DCM、EFE等,彩色多普勒超声心动图在该病的诊断及鉴别诊断中具有重要价值。小儿NVM尚缺乏有效的治疗方法,预后差。
Objective To investigate the clinical features of noncompaction of ventricular myocardium（NVM） in children in order to improve the diagnosis and treatment ＇of NVM. Method A retrospective review of 25 pediatric patients with NVM was performed,who were hospitalized in Wuhan Children＇s Hospital between Jan. 2005 and Dec. 2010. Results Cardiac dysfunction was present in 19 cases（76% ） ,while arrhythmia in 7 cases（28% ） ,among the 7 cases,ventricular arrhythmia were in 4 cases（ 16% ）. Endocardial thrombosis was not found in any of these cases. The typical change of NVM was spotted in all 25 cases through the color doppler echocardiography. Left ventricular involvement was present in 23 cases （92%） ,right ventricular involvement in 2 cases （8%） ,and enlargement of the heart in 24 cases（96% ） ,left ventricular ejection fraction less than 50% was in 20 cases （80%）. Before hospitalization,3 cases were misdiagnosed as dilated cardiomyopathy （ DCM ）, and 1 case was misdiagnosed as endocardial fibroelastosis （EFE）. During O. 4 to 5.6 years followed up,6 cases died of severe heart failure, 1 case had sudden death probably due to severe arrhythmia, while 4 cases failed to follow up. Conclusions The main clinical manifestations of NVM in children are cardiac dysfunction and ventricular arrhythmias. NVM can easily be misdiagnosed as DCM and EFE in children. Color doppler echocardiography has great value in the diagnosis and differential diagnosis of NVM. Due to the lack of effectual treatment, theprognosis of NVM in children is poor.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2012年第1期32-33,共2页
Journal of Applied Clinical Pediatrics
关键词
心肌致密化不全
心肌病
彩色多普勒超声心动图
儿童
noncompaction of ventricular myocardium
cardiomyopathy
color doppler echocardiography
child
