Glavinovic Tamara, Cheung David Y C, Cordova Perez Francisco J, Soni Anita, Memauri Brett, Jassal Davinder S
Institute of Cardiovascular Sciences, St, Boniface Research Centre, University of Manitoba, Winnipeg, Manitoba, Canada.
BMC Res Notes. 2014 Nov 5;7:787. doi: 10.1186/1756-0500-7-787.
Left ventricular non compaction is a relatively rare congenital disorder characterized by prominent trabeculations and intertrabecular recesses with the potential for thromboembolism, arrhythmias, and sudden cardiac death as adverse effects. Echocardiography has traditionally been employed as the primary mode of imaging; however, with the advent of cardiac magnetic resonance as a more precise imaging technique, the disorder known as left ventricle non compaction is becoming more broadly defined with increasing recognition of right ventricle (RV) involvement.
This report describes a 52-year-old Caucasian female with new onset atrial fibrillation with an unusual finding of left ventricular non compaction and right ventricular dysfunction on transthoracic echocardiogram with preserved left ventricular ejection fraction. Cardiac magnetic resonance imaging demonstrated a disproportionately affected right ventricle, with apical free wall dyskinesis.
This case illustrates the unique occurrence of left ventricular non compaction with preserved ejection fraction alongside RV free wall dyskinesis and RV systolic dysfunction. The significance of this is yet unknown given the paucity of existing literature. This report serves to highlight the vast heterogeneity within left ventricular non compaction as we are better able to delineate this disorder using increasingly sophisticated imaging techniques.
左心室心肌致密化不全是一种相对罕见的先天性疾病,其特征为显著的肌小梁和小梁间隐窝,可能会导致血栓栓塞、心律失常和心源性猝死等不良反应。传统上,超声心动图一直是主要的成像方式;然而,随着心脏磁共振成像这一更为精确的成像技术的出现,随着对右心室(RV)受累的认识不断增加,左心室心肌致密化不全这种疾病正得到更广泛的定义。
本报告描述了一名52岁的白种女性,新发房颤,经胸超声心动图显示左心室心肌致密化不全和右心室功能障碍,左心室射血分数保留,这一发现不同寻常。心脏磁共振成像显示右心室受累程度不成比例,心尖游离壁运动障碍。
本病例说明了左心室心肌致密化不全伴射血分数保留,同时伴有右心室游离壁运动障碍和右心室收缩功能障碍的独特情况。鉴于现有文献较少,其意义尚不清楚。本报告旨在强调随着我们能够使用日益复杂的成像技术更好地描绘这种疾病,左心室心肌致密化不全存在巨大的异质性。