Abstract:
For several years, left ventricular non-compaction (LVNC) was considered as a true cardiomyopathy and several definitions have followed one another. Particularly, LVNC was characterized by prominent left ventricular trabeculae separated from deep intertrabecular recesses. Several echocardiographic criteria and cardiac magnetic resonance imaging (CMR) criteria have been used to diagnose LVNC, leading to overestimate the diagnosis of LVNC in patients with other diseases and/or physiological conditions. Left ventricular hypertrabeculation (LVH) can be present in several cardiac diseases and physiological conditions: heart failure with reduced ejection fraction, thalassemia and other hematological diseases, pregnancy, athlete\'s heart. Thus, the presence of LVH does not necessarily indicate the presence of an LVNC. In addition, the great heterogeneity of clinical manifestations has raised concerns regarding the existence of a true LVNC as a cardiomyopathy. In fact, LVNC ranges from genetic to acquired and even transient conditions, isolated forms or forms associated with other cardiomyopathies, congenital heart diseases or syndromes with a very different prognosis. Thus, considering LVH as a manifestation of various diseases and physiological conditions, the recent 2023 ESC guidelines on cardiomyopathies did not include LVNC among cardiomyopathies, but they suggested using the term \"LVH\" rather than LVNC, to describe this phenotype especially when it is transient or of adult-onset. In this review, we aimed to make an excursion on LVNC, from its initial description to the present day, to understand why current guidelines decided to consider LVH as a phenotypic trait rather than a distinct cardiomyopathy.
摘要:
几年来,左心室压缩不全(LVNC)被认为是一种真正的心肌病,并且有几个定义相继出现.特别是,LVNC的特征是突出的左心室小梁与深层小梁间凹陷分离。一些超声心动图标准和心脏磁共振成像(CMR)标准已用于诊断LVNC,导致高估患有其他疾病和/或生理状况的患者的LVNC诊断。左心室过度扩张(LVH)可存在于多种心脏疾病和生理状况中:射血分数降低的心力衰竭,地中海贫血和其他血液系统疾病,怀孕,运动员的心。因此,LVH的存在并不一定表示LVNC的存在。此外,临床表现的巨大异质性引起了人们对真正的LVNC作为心肌病存在的担忧.事实上,LVNC的范围从遗传到获得性甚至瞬时条件,孤立的形式或与其他心肌病相关的形式,先天性心脏病或预后非常不同的综合征。因此,考虑到LVH是各种疾病和生理状况的表现,最近的2023年ESC心肌病指南没有包括LVNC在心肌病中,但他们建议使用术语“LVH”而不是LVNC,描述这种表型,特别是当它是短暂的或成人发作时。在这次审查中,我们的目标是在LVNC上游览,从最初的描述到现在,了解为什么当前的指南决定将LVH视为一种表型特征而不是独特的心肌病。