A DOUBLE DOSE OF AORTIC STENOSIS: AN UNUSUAL CASE.

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.