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左心室心肌致密化不全

[Noncompaction of the left ventricular myocardium].

作者信息

Saeed Sahrai, Vegsundvåg Johnny, Lode Inge

机构信息

Medisinsk avdeling, Alesund sjukehus, 6026 Alesund.

出版信息

Tidsskr Nor Laegeforen. 2009 May 28;129(11):1104-7. doi: 10.4045/tidsskr.08.0077.

DOI:10.4045/tidsskr.08.0077
PMID:19488092
Abstract

BACKGROUND

Noncompaction of the left ventricular myocardium is recently described as a cause of left ventricular dysfunction. In the article, we describe epidemiological and clinical aspects of this condition, which presents clinically at any age.

MATERIAL AND METHODS

This article is based on a review of articles from our own literature archive and relevant references in these articles.

RESULTS

Ventricular noncompaction results from an arrest in the normal endomyocardial embryogenesis, and often leads to heart failure, thrombo-embolic events and/or ventricular arrhythmias. The disorder is diagnosed by two-dimensional echocardiography or magnetic resonance imaging of the heart. The changes are typically seen in the apex and distal and middle segments of the inferior and lateral walls of the left ventricle. The affected segments of the myocardium have a two-layered structure: a compacted thin epicardial layer and an endocardial layer consisting of a prominent trabecular meshwork with deep intertrabecular spaces. The condition can be isolated with or without extracardiac disorders, or can be associated with other cardiac malformations.

INTERPRETATION

It is essential not to miss the findings of noncompaction, as the condition may lead to serious heart failure, thrombo-embolic events, ventricular tachyarrythmias or death. Early recognition of noncompaction may give better follow-up and management of patients with this condition.

摘要

背景

左心室心肌致密化不全最近被描述为左心室功能障碍的一个原因。在本文中,我们描述了这种在任何年龄均可临床呈现的病症的流行病学和临床特征。

材料与方法

本文基于对我们自己文献档案中的文章以及这些文章中的相关参考文献的综述。

结果

心室致密化不全是由于正常心内膜胚胎发育停滞所致,常导致心力衰竭、血栓栓塞事件和/或室性心律失常。该病症通过心脏二维超声心动图或磁共振成像诊断。这些改变通常见于左心室心尖部以及下壁和侧壁的远端及中间节段。心肌的受累节段具有双层结构:一层致密的薄心外膜层和一层由突出的小梁网状结构及深陷的小梁间隙组成的心内膜层。该病症可孤立存在,伴或不伴有心外疾病,也可与其他心脏畸形相关。

解读

务必不能漏诊致密化不全的表现,因为该病症可能导致严重心力衰竭、血栓栓塞事件、室性快速心律失常或死亡。早期识别致密化不全可为患有该病症的患者提供更好的随访和管理。

相似文献

1
[Noncompaction of the left ventricular myocardium].左心室心肌致密化不全
Tidsskr Nor Laegeforen. 2009 May 28;129(11):1104-7. doi: 10.4045/tidsskr.08.0077.
2
Isolated noncompaction of the left ventricular myocardium -- a review of the literature two decades after the initial case description.孤立性左心室心肌致密化不全——首例病例描述二十年后的文献综述
Clin Res Cardiol. 2007 Jul;96(7):481-8. doi: 10.1007/s00392-007-0528-6. Epub 2007 Jun 4.
3
Myocardial necrosis in both left and right ventricles detected by delayed-enhanced magnetic resonance imaging in a patient with isolated noncompaction of the ventricular myocardium.心脏磁共振延迟增强扫描发现孤立性左、右心室心肌致密化不全患者的心肌坏死。
Int J Cardiol. 2009 Apr 17;133(3):e94-6. doi: 10.1016/j.ijcard.2007.11.041. Epub 2008 Jan 10.
4
The noncompaction of the left ventricular myocardium: our paediatric experience.左心室心肌致密化不全:我们的儿科经验
J Cardiovasc Med (Hagerstown). 2007 Nov;8(11):904-8. doi: 10.2459/JCM.0b013e32801462b0.
5
Left ventricular noncompaction-a rare cause of triad: heart failure, ventricular arrhythmias, and systemic embolic events: a case report.左心室心肌致密化不全——三联征(心力衰竭、室性心律失常和全身栓塞事件)的罕见病因:病例报告。
J Med Case Rep. 2021 Jun 8;15(1):316. doi: 10.1186/s13256-021-02862-x.
6
Isolated noncompaction of the ventricular myocardium. A study in an adult male and literature review.孤立性心室心肌致密化不全:一例成年男性病例研究及文献综述
Ital Heart J. 2000 May;1(5):372-5.
7
Isolated noncompaction of the ventricular myocardium.孤立性心室心肌致密化不全
Turk J Pediatr. 2002 Jan-Mar;44(1):83-5.
8
Xq28-linked noncompaction of the left ventricular myocardium: prenatal diagnosis and pathologic analysis of affected individuals.Xq28连锁的左心室心肌致密化不全:患病个体的产前诊断及病理分析
Am J Med Genet. 1997 Oct 31;72(3):257-65.
9
Cardiac resynchronization and cardioverter defibrillation therapy in a patient with isolated noncompaction of the ventricular myocardium.孤立性心室心肌致密化不全患者的心脏再同步化及心脏转复除颤治疗
Int J Cardiol. 2009 Aug 21;136(3):e66-8. doi: 10.1016/j.ijcard.2008.05.022. Epub 2008 Jul 31.
10
[Echocardiography and magnetic resonance tomography of the heart in diagnosis of noncompaction of left ventricular myocardium].[心脏超声心动图和磁共振断层扫描在左心室心肌致密化不全诊断中的应用]
Kardiologiia. 2009;49(4):25-8.

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Cardiology. 2025 Jan 7:1-15. doi: 10.1159/000543170.
2
Left ventricular non-compaction and its cardiac and neurologic implications.左心室心肌致密化不全及其心脏和神经系统表现。
Heart Fail Rev. 2010 Nov;15(6):589-603. doi: 10.1007/s10741-010-9175-5.