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心肌致密化不全(noncompaction of ventricular myocardium,NVM)又称海绵状心肌,大部分患者以左心室心肌致密化不全(left ventricular noncompaction,LVNC)为主,是胚胎期心内膜肌小梁致密化过程异常终止,形成以大量突出的肌小梁和深陷的小梁隐窝为特征的心肌病。该病发病年龄从1.5~75岁不等。随着影像学诊断技术的进步,临床医师对该病的认识水平不断提高,NVM的检出率也逐年增加,但NVM确切的病因及其发病机制尚未明确。本文将着重从与NVM相关的发病基因进行
Myocardial densification incomplete (noncompaction of ventricular myocardium, NVM), also known as cavernous myocardium, most patients with left ventricular myocardial densification imperfect (LVNC) mainly embryonic endocardial trabecular densification process Abnormal termination, the formation of a large number of prominent trabecular and trabecular recession is characterized by cardiomyopathy. The age of onset of the disease from 1.5 to 75 years old. With the progress of imaging diagnostic techniques, clinicians have been increasing awareness of the disease, NVM detection rate also increased year by year, but the exact etiology of NVM and its pathogenesis is not yet clear. This article will focus on genes associated with NVM