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左心室心肌致密化不全:心脏移植病例

Left ventricular noncompaction: case of a heart transplant.

作者信息

Tigen Kursat, Karaahmet Tansu, Kahveci Gokhan, Mutlu Bülent, Basaran Yelda

机构信息

Kartal Kosuyolu Heart Education and Research Hospital, Cardiology Department, Istanbul, Turkey.

出版信息

Eur J Echocardiogr. 2008 Jan;9(1):126-9. doi: 10.1016/j.euje.2007.04.007.

Abstract

Noncompaction of the ventricular myocardium (NVM) is a rare congenital cardiomyopathy characterized by multiple prominent trabeculations with deep intertrabecular recesses resulting from an arrest in normal embryogenesis of the endocardium and myocardium. The major clinical manifestations are depressed left ventricular systolic and diastolic function, systemic embolism, ventricular tachyarrhythmias, conduction disorders and neurologic abnormalities. We present a 21-year-old female who was diagnosed as dilated cardiomyopathy due to isolated noncompaction of the left ventricle and underwent cardiac transplantation.

摘要

心室心肌致密化不全(NVM)是一种罕见的先天性心肌病,其特征是多个突出的肌小梁以及肌小梁间深陷的隐窝,这是由于心内膜和心肌正常胚胎发育停滞所致。主要临床表现为左心室收缩和舒张功能降低、全身栓塞、室性快速心律失常、传导障碍和神经学异常。我们报告一例21岁女性患者,因孤立性左心室致密化不全被诊断为扩张型心肌病,并接受了心脏移植。

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