左心室心肌致密化不全心肌病的研究进展

Research progress of left ventricular non-compaction cardiomyopathy

左心室心肌致密化不全心肌病(LVNC)以突出的骨小梁、小梁隐窝以及左室心肌等表达紊乱为特点,主要表现为左室心尖处不正常的肌小梁形成,可合并左心室扩张,伴收缩、舒张功能不全,或合并各种形式的先天性心脏病,并会导致左心室、右心室或双心室的心力衰竭。本文就LVNC病的发病机制、流行病学、临床表现、诊断、治疗和预后进行综述。

Left ventricular non-compaction cardiomyopathy（LVNC）is characterised by prominent trabeculae,intratrabecular recesses,and left ventricular myocardium abnormity,most frequently at the apex presenting with abnormal trabeculae.It can be associated with left ventricular dilation,systolic or diastolic dysfunction,or various forms of congenital heart disease.It can result in left heart failure,right heart failure or whole heart failure.The article focuses on the pathogenesis,epidemiology,clinical feature,diagnosis and prognosis of LVNC.