一种罕见的心肌病类型：左心室心肌致密化不全心肌病。
A rare form of cardiomyopathy: left ventricular non-compaction cardiomyopathy.
作者信息
Goud Aditya, Padmanabhan Sriram
机构信息
Department of Internal Medicine, MedStar Franklin Square Medical Center, Baltimore, MD, USA;
Department of Cardiology, MedStar Franklin Square Medical Center, Baltimore, MD, USA.
出版信息
J Community Hosp Intern Med Perspect. 2016 Feb 17;6(1):29888. doi: 10.3402/jchimp.v6.29888. eCollection 2016.
Left ventricular non-compaction is a recently recognized, rare form of cardiomyopathy. It is based on the arrest of endomyocardial morphogenesis during embryogenesis. It was first described in 1984 by Engberding who described it as isolated 'sinusoids' within the LV. Right now its prevalence is estimated at 0.014 to 1.3 and 3-4% in heart failure patients. Its clinical manifestations are highly variable, ranging from no symptoms to disabling congestive heart failure, arrhythmias, and systemic thromboemboli. Doppler Echocardiogram is considered the diagnostic procedure of choice and treatment is symptomatic management of its symptoms and complications.
左心室心肌致密化不全是一种最近才被认识到的罕见心肌病形式。它基于胚胎发育过程中心内膜形态发生的停滞。1984年恩格伯丁首次对其进行描述，他将其描述为左心室内孤立的“血窦”。目前估计其患病率在普通人群中为0.014%至1.3%，在心力衰竭患者中为3%至4%。其临床表现高度多变，从无症状到导致失能的充血性心力衰竭、心律失常和全身性血栓栓塞。多普勒超声心动图被认为是首选的诊断方法，治疗是对其症状和并发症进行对症处理。
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