Malla R, Sharma R, Rauniyar B, Kc M B, Maskey A, Joshi D, Hamal S
Department of Cardiology, Shahid Gangalal National Heart Center, Bansbari, Kathmandu, Nepal.
JNMA J Nepal Med Assoc. 2009 Apr-Jun;48(174):180-4.
Left Ventricular Noncompaction (LVNC) is a genetic cardiac disease of emerging importance with a distinct clinical and pathophysiological presentation. The most common clinical manifestations include heart failure, arrhythmias and embolic events, and in children it may be associated with facial dysmorphisms and Wolff-Parkinson-White syndrome. The diagnosis of LVNC, however, is often missed, most often as a consequence of ignorance of the condition. Echocardiography is considered the reference standard for the diagnosis of LVNC. Prognosis remains poor for patients with impaired systolic left ventricular function, as treatment options are very limited. Because of the familial association of LVNC, first-degree relatives should be screened by Echocardiography.
左心室心肌致密化不全（LVNC）是一种新出现的具有重要意义的遗传性心脏病，具有独特的临床和病理生理表现。最常见的临床表现包括心力衰竭、心律失常和栓塞事件，在儿童中可能与面部畸形和预激综合征有关。然而，LVNC的诊断常常被漏诊，多数情况是由于对该病不了解。超声心动图被认为是LVNC诊断的参考标准。对于左心室收缩功能受损的患者，预后仍然很差，因为治疗选择非常有限。由于LVNC具有家族相关性，一级亲属应接受超声心动图筛查。
