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目的探讨小儿心室肌致密化不全(NVM)的临床特点,提高对本病的认识。方法对2例 NVM 患儿的发病情况、临床表现、心电图与超声心动图等资料与随访结果进行分析和总结。结果 2例均为男孩,1例症状少,而另1例症状明显;2例心电图均表现有心肌缺血和多种心律失常:心房扑动、心房纤颤、室上性心动过速、房室传导阻滞、短阵性室性心动过速等。超声心动图(UCG)特异性表现为心室内有过多突起的肌小梁,彩色多普勒血流显像(CDFI)证实有无数与心室腔交通的深陷的肌小梁间隙。随访3年,1例心功能明显改善,另1例死亡。结论小儿 NVM 的临床表现主要有心功能不全和心律失常,UCG 是诊断 NVM 的重要方法。早期诊断与治疗可改善预后。
Objective To investigate the clinical features of pediatric ventricular myocardial degeneration (NVM) and to improve the understanding of this disease. Methods The incidence, clinical manifestations, electrocardiogram (ECG) and echocardiography in 2 children with NVM were analyzed and summarized. Results 2 cases were boys, 1 case had less symptoms and 1 case had obvious symptoms. 2 cases of ECG showed myocardial ischemia and various arrhythmias: atrial flutter, atrial fibrillation, supraventricular tachycardia, Ventricular conduction block, paroxysmal ventricular tachycardia and so on. Echocardiography (UCG) is specifically characterized by trabecular hyperextension in the ventricles, and deep pleurodesis of the trabecular spaces confirmed by color Doppler flow imaging (CDFI). After 3 years of follow-up, one patient’s cardiac function improved significantly and the other one died. Conclusion The clinical manifestations of pediatric NVM mainly include cardiac insufficiency and arrhythmia. UCG is an important method for the diagnosis of NVM. Early diagnosis and treatment can improve the prognosis.