Li Qing, Miao Lianjie, Xia Lihong, Abdelnasser Hala Y, Zhang Fang, Lu Yangyang, Nusrat Anika, Tabassum Mantasha, Li Juxiang, Wu Mingfu
Department of Cardiovascular Medicine, The Second Affiliated Hospital of Nanchang University, Nanchang 330006, China.
Department of Molecular and Cellular Physiology, Albany Medical College, Albany, NY 12208, USA.
J Cardiovasc Dev Dis. 2022 Feb 2;9(2):49. doi: 10.3390/jcdd9020049.
Left ventricular noncompaction (LVNC) is a type of cardiomyopathy characterized anatomically by prominent ventricular trabeculation and deep intertrabecular recesses. The mortality associated with LVNC ranges from 5% to 47%. The etiology of LVNC is yet to be fully understood, although decades have passed since its recognition as a clinical entity globally. Furthermore, critical questions, i.e., whether LVNC represents an acquired pathology or has a congenital origin and whether the reduced contractile function in LVNC patients is a cause or consequence of noncompaction, remain to be addressed. In this study, to answer some of these questions, we analyzed the clinical features of LVNC patients. Out of 9582 subjects screened for abnormal cardiac functions, 45 exhibit the characteristics of LVNC, and 1 presents right ventricular noncompaction (RVNC). We found that 40 patients show valvular regurgitation, 39 manifest reduced systolic contractions, and 46 out of the 46 present different forms of arrhythmias that are not restricted to be caused by the noncompact myocardium. This retrospective examination of LVNC patients reveals some novel findings: LVNC is associated with regurgitation in most patients and arrhythmias in all patients. The thickness ratio of the trabecular layer to compact layer negatively correlates with fractional shortening, and reduced contractility might result from LVNC. This study adds evidence to support a congenital origin of LVNC that might benefit the diagnosis and subsequent characterization of LVNC patients.
左心室心肌致密化不全（LVNC）是一种心肌病，其解剖学特征为显著的心室肌小梁和深陷的小梁间隐窝。与LVNC相关的死亡率在5%至47%之间。尽管自其在全球被确认为一种临床实体以来已经过去了几十年，但LVNC的病因仍未完全明确。此外，一些关键问题，即LVNC是一种后天性病变还是先天性起源，以及LVNC患者收缩功能降低是心肌致密化不全的原因还是结果，仍有待解决。在本研究中，为了回答其中一些问题，我们分析了LVNC患者的临床特征。在9582名接受心脏功能异常筛查的受试者中，45人表现出LVNC的特征，1人表现为右心室心肌致密化不全（RVNC）。我们发现40例患者存在瓣膜反流，39例表现为收缩期收缩功能降低，46例患者出现不同形式的心律失常，且不限于由致密化不全心肌引起。对LVNC患者的这项回顾性研究揭示了一些新发现：LVNC在大多数患者中与反流相关，在所有患者中与心律失常相关。小梁层与致密层的厚度比与缩短分数呈负相关，收缩功能降低可能是由LVNC导致的。本研究为支持LVNC的先天性起源增加了证据，这可能有助于LVNC患者的诊断及后续特征描述。
