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Clinical Analysis of 25 Children with Noncompaction of Ventricular Myocardium

DENG Shu-zhen,SUN Dong-ming,WANG Rui-geng,LIU Ling,XIA Kun,PENG Jing(Department of Cardiology,Wuhan Children′s Hospital,Wuhan 430016,Hubei Province,China)  
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 ventri-cular 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 0.4 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,the prognosis of NVM in children is poor.
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