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左心室心肌致密化不全与逆向重构的罕见病症

The Rare Condition of Left Ventricular Non-Compaction and Reverse Remodeling.

作者信息

Bustea Cristiana, Bungau Alexa Florina, Tit Delia Mirela, Iovanovici Diana Carina, Toma Mirela Marioara, Bungau Simona Gabriela, Radu Andrei-Flavius, Behl Tapan, Cote Adrian, Babes Elena Emilia

机构信息

Department of Preclinical Disciplines, Faculty of Medicine and Pharmacy, University of Oradea, 410073 Oradea, Romania.

Doctoral School of Biomedical Sciences, University of Oradea, 410087 Oradea, Romania.

出版信息

Life (Basel). 2023 Jun 3;13(6):1318. doi: 10.3390/life13061318.

Abstract

Left ventricular non-compaction (LVNC) is a rare disease defined by morphological criteria, consisting of a two-layered ventricular wall, a thin compacted epicardial layer, and a thick hyper-trabeculated myocardium layer with deep recesses. Controversies still exist regarding whether it is a distinct cardiomyopathy (CM) or a morphological trait of different conditions. This review analyzes data from the literature regarding diagnosis, treatment, and prognosis in LVNC and the current knowledge regarding reverse remodeling in this form of CM. Furthermore, for clear exemplification, we report a case of a 41-year-old male who presented symptoms of heart failure (HF). LVNC CM was suspected at the time of transthoracic echocardiography and was subsequently confirmed upon cardiac magnetic resonance imaging. A favorable remodeling and clinical outcome were registered after including an angiotensin receptor neprilysin inhibitor in the HF treatment. LVNC remains a heterogenous CM, and although a favorable outcome is not commonly encountered, some patients respond well to therapy.

摘要

左心室心肌致密化不全(LVNC)是一种罕见疾病,由形态学标准定义,包括两层心室壁,一层薄的致密的心外膜层,以及一层厚的、有深陷隐窝的心肌小梁增多层。关于它是一种独特的心肌病(CM)还是不同病症的形态学特征,仍存在争议。本综述分析了文献中有关LVNC的诊断、治疗和预后的数据,以及关于这种CM形式的逆向重构的现有知识。此外,为了清晰举例,我们报告了一例41岁男性出现心力衰竭(HF)症状的病例。经胸超声心动图检查时怀疑为LVNC CM,随后心脏磁共振成像确诊。在HF治疗中加入血管紧张素受体脑啡肽酶抑制剂后,出现了良好的重构和临床结果。LVNC仍然是一种异质性CM,虽然通常不会有良好的结果,但一些患者对治疗反应良好。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1267/10305066/c80d4b246714/life-13-01318-g001.jpg

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