小儿心肌致密化不全123例临床分析

摘要：目的　探讨123例小儿心肌致密化不全（NVM）的临床特点。方法　2005年4月至2009年9月广东省心血管病研究所收治NVM患儿123例，对所有患儿临床表现、心电图、X线胸片、超声心动图和其中31例心导管检查、24例心脏CT检查的结果进行分析和总结。结果　心前区不适、气促、多汗50例，心脏扩大110例，心音低钝30例；合并其他心脏畸形65例；心电图ST-T改变104例，心律失常29例；心胸比例大于0.5者98例；超声心动图均见受累心肌肌小梁过度隆突，形成隐窝，成网状结构；行心脏CT或心导管检查的患儿均有相应的特征性影像学改变。结论　小儿NVM临床主要表现为心功能不全和心律失常，各系统栓塞罕见；易合并其他心脏畸形；超声心动图是诊断NVM的首选方法。

Clinical analysis of noncompaction of ventricular myocardium in 123 children.

Abstract： Objective　To explore the clinical features of noncompaction of the ventricular myocardium（NVM） in 123 children. Methods　Clinical manifestation， electrocardiogram， chest X-ray and echocardiogram of 123 patients as well as 31 cases of cardiac catheterization and 24 of cardiac CT of NVM were studied. Results　Among 123 patients， 50 suffered from chest discomfort， short breath and much sweat， 110 were cardiac dilatation， and a decrease of the intensity of heart sounds was found in 30 children； 65 coexisted with cardiac abnormalities；104 patients had ST-T change on electrocardiogram. Cardiac arrhythmia was found in 29 cases； 98 cases of cardiothoracic ratio > 0.5 were noticed in chest X-ray. Echocardiograph examination showed that numerous prominent ventricular trabeculations and deep intertrabecular recesses were presented in ventricle. Characteristic changes of image in cardiac catheterization and cardiac CT were found as well. Conclusion　Clinical features of NVM in children are a depressed cardiac function and arrhythmia， an infrequency of systemic embolization with a higher rate of coexistence of cardiac abnormalities. Echocardiography is a reliable promising diagnostic tool for NVM.