LEFT VENTRICULAR NONCOMPACTION CARDIOMYOPATHY: A SCOPING REVIEW.

Annals of Ibadan postgraduate medicine Pub Date : 2023-08-01 Epub Date: 2023-11-01
O S Ogah, E P Iyawe, K F Okwunze, C A Nwamadiegesi, F E Obiekwe, M O Fabowale, M Okeke, O A Orimolade, O V Olalusi, A Aje, A Adebiyi
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Abstract

Introduction: There has been an upsurge in the reporting of cases of Left Ventricular Noncompaction (LVNC) cardiomyopathy in medical literature in the last 35 years due to advances in medical imaging.The condition was first described in 1926 and the first reported case by echocardiography was in 1984. The American Heart Association considers LVNC a primary cardiomyopathy of genetic origin, while the European Society of Cardiology and the World Health Organization grouped it as an unclassified cardiomyopathy. Its variability in terms of genetic profile, phenotypic expression, clinical presentation, and histopathological findings makes it somewhat a variant of other cardiomyopathies.

Case presentation: Patients with LVNC cardiomyopathy may not have any symptoms or may present with ventricular arrhythmias, heart failure, thromboembolism, or sudden death. LVNC cardiomyopathy diagnosis is typically made by echocardiography, although there are higher resolution cardiac imaging techniques. Management will depend on the patient's clinical presentation. Due to its genetic association, there is a need to screen living relatives once the diagnosis is made in an individual.

Conclusion: The aim of this paper is to review current knowledge on this condition.

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左室非充盈性心肌病:范围综述。
导言:过去 35 年来,由于医学影像技术的进步,医学文献中关于左心室非充盈性(LVNC)心肌病的病例报道激增。该病于 1926 年首次被描述,1984 年首次报道超声心动图病例。美国心脏协会认为 LVNC 是一种遗传性原发性心肌病,而欧洲心脏病学会和世界卫生组织则将其归类为未分类心肌病。LVNC 在遗传特征、表型表达、临床表现和组织病理学发现等方面的变异性使其在某种程度上成为其他心肌病的变种:LVNC 心肌病患者可能没有任何症状,也可能出现室性心律失常、心力衰竭、血栓栓塞或猝死。尽管有分辨率更高的心脏成像技术,但 LVNC 心肌病的诊断通常是通过超声心动图做出的。治疗方法取决于患者的临床表现。由于该病与遗传有关,一旦确诊患者,需要对其在世亲属进行筛查:本文旨在回顾目前对这种疾病的认识。
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