Abstract：
Supravalvular aortic stenosis is a rare congenital anomaly (less than 0.05% of all congenital heart defects). This aortic root anomaly consists in a narrow aortic lumen immediately above the aortic valve and represents the least common form of left ventricular outflow tract obstruction. Clinical presentation is usually in the first decades of life. In most cases, the aortic valve leaflets are morphologically normal. However, aortic insufficiency due the high systolic pressure proximal to the sinotubular junction is the most commonly abnormality described. There are very few cases described in the literature with concomitant valvular and supra-valvular aortic stenosis.
摘要：
主动脉瓣上狭窄是一种罕见的先天性异常（小于所有先天性心脏缺陷的0.05％）。这种主动脉根部异常存在于主动脉瓣正上方狭窄的主动脉腔中，是最不常见的左心室流出道阻塞形式。临床表现通常在生命的最初几十年。在大多数情况下,主动脉瓣小叶形态正常。然而,由于窦管交界处的高收缩压引起的主动脉瓣关闭不全是最常见的异常。文献中描述的伴有瓣膜和瓣膜上主动脉瓣狭窄的病例很少。
