Abstract
左室心尖发育不良(left ventricular apical hypoplasia,LVAH)是一种极少见的左室心肌病变,主要表现为左室心尖部缺如呈球形缩短,目前多认为属于先天性畸形.FernandezValls等[1]2004年在一篇放射学论文中首次描述了这种畸形,此后,一些磁共振、CT及超声心动图诊断的病例陆续发表,国内关于左室心尖发育不良报道极少,为促进对其更多的了解,现对此疾病作一综述.一、病理解剖学及临床LVAH的发生机制不明.人体胚胎期第3周开始心脏发育,出现原始心管,进而形成原始心腔.胚胎期第5周,肌性室间隔形成,原始心室开始左右分侧.本病可能系原始心室左右分侧时左室发育不充分,随着肌性室间隔的持续生长,正常发育的右室相对延长环绕缩短的左室心尖,导致本病形成n]。
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