Krankenanstalt Rudolfstiftung, Vienna, Danube University Krems, Krems, Postfach 20, 1180, Vienna, Austria.
Heart Fail Rev. 2010 Nov;15(6):589-603. doi: 10.1007/s10741-010-9175-5.
Left ventricular non-compaction, also known as left ventricular hypertrabeculation (LVHT), is a morphological abnormality of the left ventricular myocardium, characterised by a meshwork of myocardial strings, interlacing, and orderless in arrangement. LVHT is most frequently located in the apex and the lateral wall and may occur with or without other congenital or acquired cardiac abnormalities. LVHT is believed to be congenital in the majority of the cases but may develop during life in single cases (acquired LVHT). Congenital LVHT is believed to result from defective late-stage embryonic development of the myocardial architecture. The pathogenesis of acquired LVHT remains speculative. LVHT is most frequently found on transthoracic echocardiography and cardiac MRI but may be visualised also with other imaging techniques. In the majority of the cases, LVHT is associated with hereditary cardiac, neuromuscular, non-cardiac/non-muscle disease, or chromosomal aberrations. In the majority of the cases, LVHT is complicated by ventricular arrhythmias, systolic dysfunction, cardiac embolism, or sudden cardiac death. LVHT per se does not require a specific treatment. Only in case of complications, such as ventricular arrhythmias, cardioembolism, or systolic dysfunction, adequate therapy is indicated. Though initially assessed as poor, the prognosis of LVHT has meanwhile improved, most likely due to the increased awareness for the abnormality and the timely administration of adequate therapy.
左心室心肌致密化不全，又称左心室心肌肥厚（LVHT），是一种左心室心肌的形态学异常，其特征为心肌条索交织、排列无序的网格状结构。LVHT 最常发生于心尖部和侧壁，可伴有或不伴有其他先天性或后天性心脏异常。大多数情况下，LVHT 被认为是先天性的，但在个别情况下也可能在生命过程中发生（获得性 LVHT）。先天性 LVHT 被认为是心肌结构晚期胚胎发育缺陷的结果。获得性 LVHT 的发病机制仍存在推测。LVHT 最常通过经胸超声心动图和心脏 MRI 发现，但也可以通过其他成像技术观察到。在大多数情况下，LVHT 与遗传性心脏、神经肌肉、非心脏/非肌肉疾病或染色体异常有关。在大多数情况下，LVHT 并发室性心律失常、收缩功能障碍、心源性栓塞或心源性猝死。LVHT 本身不需要特定的治疗。只有在出现并发症（如室性心律失常、心源性栓塞或收缩功能障碍）时，才需要进行适当的治疗。尽管最初评估预后较差，但 LVHT 的预后已得到改善，这可能是由于对该异常的认识提高以及及时给予适当的治疗。
