左心室非充盈性心肌病是怎么回事?是或不是:这是一个问题。

IF 3 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Current Problems in Cardiology Pub Date : 2024-08-11 DOI:10.1016/j.cpcardiol.2024.102787
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引用次数: 0

摘要

数年来,左心室不充盈(LVNC)一直被认为是一种真正的心肌病,并且有多种定义相继出现。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)标准被用于诊断 LVNC,导致患有其他疾病和/或生理状况的患者被高估了 LVNC 的诊断。左心室肥厚(LVH)可出现在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病、妊娠、运动员心脏。因此,LVH 的存在并不一定意味着 LVNC 的存在。此外,临床表现的巨大异质性也引起了人们对是否存在真正的 LVNC 心肌病的担忧。事实上,LVNC 的表现形式多种多样,既有遗传性的,也有后天获得性的,甚至还有一过性的,既有孤立的,也有与其他心肌病、先天性心脏病或预后大相径庭的综合征相关的表现形式。因此,考虑到 LVH 是各种疾病和生理状况的一种表现形式,最近的 2023 年 ESC 心肌病指南并未将 LVNC 纳入心肌病,但建议使用 "LVH "而非 LVNC 来描述这种表型,尤其是一过性或成人发病的 LVH。在这篇综述中,我们旨在对 LVNC 从最初的描述到今天的发展进行梳理,以了解为什么目前的指南决定将 LVH 视为一种表型特征,而不是一种独特的心肌病。
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What happened to the left ventricular non-compaction cardiomyopathy? to be or not to be: This is the question

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.

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来源期刊
Current Problems in Cardiology
Current Problems in Cardiology 医学-心血管系统
CiteScore
4.80
自引率
2.40%
发文量
392
审稿时长
6 days
期刊介绍: Under the editorial leadership of noted cardiologist Dr. Hector O. Ventura, Current Problems in Cardiology provides focused, comprehensive coverage of important clinical topics in cardiology. Each monthly issues, addresses a selected clinical problem or condition, including pathophysiology, invasive and noninvasive diagnosis, drug therapy, surgical management, and rehabilitation; or explores the clinical applications of a diagnostic modality or a particular category of drugs. Critical commentary from the distinguished editorial board accompanies each monograph, providing readers with additional insights. An extensive bibliography in each issue saves hours of library research.
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