Sun C C, Jacot J, Brenner J I
Department of Pathology, University of Maryland School of Medicine, Baltimore 21201.
Pediatr Pathol. 1992 Sep-Oct;12(5):751-9. doi: 10.3109/15513819209024230.
Supravalvular aortic stenosis (SVAS) is an uncommon congenital cardiac anomaly. We report sudden death, occurring during exercise, of a child who had SVAS with fusion of the right coronary aortic leaflet to the supravalvular aortic ridge, resulting in a closed sinus of Valsalva except for a few pinpoint fenestrations in the dysplastic leaflet. In addition, both aortic and pulmonic valves were dysplastic and stenotic. We postulate that near total isolation of the right coronary artery ostium from the aortic lumen compromised the blood supply to the hypertrophied ventricles. We emphasize the importance of other cardiac anomalies associated with SVAS as well as the development of coronary insufficiency.
瓣上主动脉狭窄（SVAS）是一种罕见的先天性心脏异常。我们报告了一名患有SVAS的儿童在运动期间突然死亡，该患儿右冠状动脉主动脉瓣叶与瓣上主动脉嵴融合，导致除发育异常的瓣叶上有几个针孔状开窗外，瓦尔萨尔瓦窦完全封闭。此外，主动脉瓣和肺动脉瓣均发育异常且狭窄。我们推测右冠状动脉口几乎完全与主动脉腔隔离，损害了肥厚心室的血液供应。我们强调与SVAS相关的其他心脏异常以及冠状动脉供血不足发展的重要性。
