Aurora Cardiovascular Services, Aurora Sinai/Aurora St. Luke's Medical Centers, University of Wisconsin School of Medicine and Public Health, 2801 W. Kinnickinnic River Parkway #845, Milwaukee, WI 53215, USA.
J Am Soc Echocardiogr. 2012 Apr;25(4):363-75. doi: 10.1016/j.echo.2011.12.023. Epub 2012 Jan 27.
Left ventricular noncompaction (LVNC) is a cardiomyopathy associated with sporadic or familial disease, the latter having an autosomal dominant mode of transmission. The clinical features associated with LVNC vary from asymptomatic to symptomatic patients, with the potential for heart failure, supraventricular and ventricular arrhythmias, thromboembolic events, and sudden cardiac death. Echocardiography is the diagnostic modality of choice, revealing the pathognomonic features of a thick, bilayered myocardium; prominent ventricular trabeculations; and deep intertrabecular recesses. Widespread use and advances in the technology of echocardiography and cardiac magnetic resonance imaging are increasing awareness of LVNC, and cardiac magnetic resonance imaging is improving the ability to stage the severity of the disease and potential for adverse clinical consequences. Study of LVNC through research in embryology, imaging, and genetics has allowed enormous strides in the understanding of this heterogeneous disease over the past 25 years.
左心室心肌致密化不全（LVNC）是一种与散发性或家族性疾病相关的心肌病，后者具有常染色体显性遗传方式。LVNC 相关的临床特征从无症状到有症状的患者不等，可能出现心力衰竭、室上性和室性心律失常、血栓栓塞事件和心源性猝死。超声心动图是首选的诊断方式，可显示出特征性的厚双层心肌、明显的心室小梁和深的小梁间隐窝。超声心动图和心脏磁共振成像技术的广泛应用和进步提高了对 LVNC 的认识，心脏磁共振成像提高了对疾病严重程度和潜在不良临床后果的分期能力。通过对胚胎学、影像学和遗传学的研究，在过去的 25 年中，对这种异质性疾病的认识取得了巨大进展。
