Left ventricular noncompaction: a distinct cardiomyopathy or a trait shared by different cardiac diseases?

Left ventricular noncompaction: a distinct cardiomyopathy or a trait shared by different cardiac diseases?
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DOI:
10.1016/j.jacc.2014.08.030
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发表时间:
2014-10-28
影响因子:
24
通讯作者:
Hall, Jennifer L.
Hall, Jennifer L.
中科院分区:
医学1区
文献类型:
--
作者:
Arbustini, Eloisa;Weidemann, Frank;Hall, Jennifer L.

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左心室致密化不全(LVNC)是一种独特的心肌病,还是不同心肌病所共有的形态特征仍存在争议。目前来自专业组织的指南推荐了诊断和治疗LVNC患者的不同策略。这篇最先进的综述讨论了导致LVNC的基本机制、临床表现和治疗方式、解剖学和病理学、胚胎学、遗传学、流行病学和成像的新见解。目前有三个标记定义LVNC:突出的左心室小梁;深的小梁间隐窝;以及较薄的致密层。虽然来自小鼠和人类的新基因数据支持LVNC是一种独特的心肌病,但不排除LVNC是一种共同的形态特征的证据。支持LVNC作为共同形态特征的标准可能取决于多个专业组织的共识指南。影像增强和遗传学应用的增加都预计将显著影响我们对LVNC的基本机制及其最佳治疗的整体理解。
Whether left ventricular noncompaction (LVNC) is a distinct cardiomyopathy or a morphologic trait shared by different cardiomyopathies remains controversial. Current guidelines from professional organizations recommend different strategies for diagnosing and treating patients with LVNC. This state-of-the-art review discusses new insights into the basic mechanisms leading to LVNC, its clinical manifestations, and treatment modalities, anatomy and pathology, embryology, genetics, epidemiology, and imaging. Three markers currently define LVNC: prominent left ventricular trabeculae; deep intertrabecular recesses; and a thin compacted layer. While new genetic data from mice and humans supports LVNC as a distinct cardiomyopathy, evidence for LVNC as a shared morphological trait is not ruled out. Criteria supporting LVNC as a shared morphological trait may depend on consensus guidelines from the multiple professional organizations. Enhanced imaging and increased use of genetics are both predicted to significantly impact our overall understanding of the basic mechanisms causing LVNC, and its optimal management.
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