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左心室致密化不全心肌病:一例病例报告及文献综述

Left ventricular noncompaction cardiomyopathy: a case report and literature review.

作者信息

Lin Ying-Nan, Wang Yu-Qiang, Yu Yi, Cao Qing, Wang Fei, Chen Shu-Yan

机构信息

Department of Geriatrics, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine Shanghai 200092, China.

Departments of Ultrasonography, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine Shanghai 200092, China.

出版信息

Int J Clin Exp Med. 2014 Dec 15;7(12):5130-3. eCollection 2014.

Abstract

Left ventricular noncompaction cardiomyopathy (LVNC) is a relatively rare congenital disorder prominently characterized by prominent trabeculations and intertrabecular recesses that communicate with the ventricular cavity rather than the coronary circulation. LVNC can occur in isolation or coexist with other cardiac and/or systemic anomalies, in especial neuromuscular disorders. The clinical presentation varies ranging from asymptomatic patients to patients who develop ventricular arrhythmias, thromboembolism, heart failure and sudden cardiac death. Although LVNC is commonly diagnosed by echocardiography, there are also other useful diagnostic techniques, including contrast ventriculography, CT and MRI. Now, it is being diagnosed more frequently in patients of all ages because of increased awareness and improvements in imaging methods. We described the case of a woman who presented with heart failure for the first time at 62 years of age. The diagnosis was LVNC. Transthoracic echocardiography showed a trabeculated, sponge-like appearance of the ventricular apical and inferolateral segments. After medical management, the patient was asymptomatic at the 1-month follow-up examination. Now we discussed the diagnosis of this case and reviewed the medical literature that pertained to LVNC.

摘要

左心室致密化不全心肌病(LVNC)是一种相对罕见的先天性疾病,其显著特征是存在粗大的肌小梁和与心室腔而非冠状动脉循环相通的肌小梁间隐窝。LVNC可单独出现,也可与其他心脏和/或全身异常并存,尤其是神经肌肉疾病。临床表现各异,从无症状患者到出现室性心律失常、血栓栓塞、心力衰竭和心源性猝死的患者都有。虽然LVNC通常通过超声心动图诊断,但也有其他有用的诊断技术,包括对比心室造影、CT和MRI。现在,由于认识的提高和成像方法的改进,各年龄段患者中LVNC的诊断越来越频繁。我们描述了一名62岁首次出现心力衰竭的女性病例。诊断为LVNC。经胸超声心动图显示心室心尖段和下外侧段呈肌小梁样、海绵状外观。经过药物治疗,患者在1个月的随访检查中无症状。现在我们讨论了该病例的诊断,并回顾了与LVNC相关的医学文献。

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