心肌致密化不全的诊断及影像学特征

心肌致密化不全(NVM)是一种少见的先天性心肌病,其发病机制与基因突变有关,具有家族遗传性。心力衰竭、心律失常及体循环栓塞是NVM的三大主要临床特点。超声心动图是影像学诊断NVM的主要方法,同时该病在MRI、CT、心室造影和核素显像上也有一些异常征象。影像学对于诊断和评价NVM具有重要临床应用和研究价值。

Diagnosis and imaging features of noncompaction of ventricular myocardium

Noncompaction of ventricular myocardium (NVM) is a rare congenital cardiomyopathy. The pathogenesis of NVM associats with gene mutation and family inheritance. Heart failure, arrhythmia and systemic embolization are the major clinical manifestations of NVM. Ultrasonic cardiography is the major diagnostic method for NVM, and abnormal features of NVM can also be observed on MRI, CT, cardiac ventriculography and radionuclide imaging. Cardiac anatomy, function and microcirculatory dysfunction can be noninvasively diagnosed and evaluated with imaging tools, which are useful for clinical application and research of NVM.