Nemes Attila
Department of Medicine, Albert Szent-Györgyi Medical School, University of Szeged, 6725 Szeged, Hungary.
J Clin Med. 2023 Dec 22;13(1):78. doi: 10.3390/jcm13010078.
Left ventricular (LV) non-compaction (LVNC) is a rare genetic cardiomyopathy due to abnormal intra-uterine arrest of compaction of the myocardial fibers during endomyocardial embryogenesis. Due to the partial or complete absence of LV compaction, the structure of the LV wall shows characteristic abnormalities, including a thin compacted epicardium and a thick non-compacted endocardium with prominent trabeculations and deep intertrabecular recesses. LVNC is frequently associated with chronic heart failure, life-threatening ventricular arrhythmias, and systemic embolic events. According to recent findings, in the presence of LVNC, dysfunctional LV proved to be associated with left atrial volumetric and functional abnormalities and consequential dilated and functionally impaired mitral annulus, partly explaining the higher prevalence of regurgitation. Although the non-compaction process morphologically affects only the LV, signs of remodeling of the right heart were also detected. Moreover, dilation and stiffening of the aorta were present. The aim of the present detailed review was to summarize findings regarding changes in cardiac mechanics, valvular abnormalities, and vascular remodeling detected in patients with LVNC.
左心室心肌致密化不全（LVNC）是一种罕见的遗传性心肌病，由于心内膜胚胎发育过程中心肌纤维致密化在子宫内异常停滞所致。由于左心室部分或完全缺乏致密化，左心室壁结构显示出特征性异常，包括薄的致密化的心外膜和厚的非致密化的心内膜，伴有明显的小梁和深陷的小梁间隐窝。LVNC常与慢性心力衰竭、危及生命的室性心律失常和全身性栓塞事件相关。根据最近的研究结果，在存在LVNC的情况下，功能失调的左心室被证明与左心房容积和功能异常以及随之而来的二尖瓣环扩张和功能受损有关，这部分解释了反流的较高患病率。尽管致密化过程在形态学上仅影响左心室，但也检测到了右心重塑的迹象。此外，还存在主动脉扩张和僵硬。本详细综述的目的是总结LVNC患者中心脏力学变化以及瓣膜异常和血管重塑方面的研究结果。
