Kurosawa H, Wagenaar S S, Becker A E
Br Heart J. 1981 Aug;46(2):211-5. doi: 10.1136/hrt.46.2.211.
A youth of 16 years of age died suddenly and quite unexpectedly while walking to school. The necropsy disclosed a quadricuspid aortic valve with complete isolation of the left coronary artery by an adherent aortic valve cusp. The left ventricular myocardium showed subendocardial contraction band necrosis suggesting that critical ischaemia had triggered a state of hypercontraction, in keeping with ventricular fibrillation as the immediate cause of death. The site of the anomaly in the aortic root showed dysplasia of the aortic wall and the affected valve cusp, histologically similar to the changes that characterise supravalvar aortic stenosis. The findings suggest a developmental anomaly, possibly a forme fruste of supravalvar aortic stenosis, rather than a postnatally acquired condition.
一名16岁的青少年在步行上学途中突然意外死亡。尸检发现为四叶式主动脉瓣，左冠状动脉被一个粘连的主动脉瓣叶完全分隔。左心室心肌显示心内膜下收缩带坏死，提示严重缺血引发了一种高收缩状态，这与心室颤动作为直接死因相符。主动脉根部的异常部位显示主动脉壁和受累瓣膜叶发育异常，组织学上类似于主动脉瓣上狭窄的特征性改变。这些发现提示是一种发育异常，可能是主动脉瓣上狭窄的一种顿挫型，而非出生后获得性疾病。
