A DOUBLE DOSE OF AORTIC STENOSIS: AN UNUSUAL CASE
DOI:
https://doi.org/10.48729/pjctvs.195Keywords:
Supravalvular aortic stenosis, aortic valve stenosis, left ventricular outflow tract obstructionAbstract
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.
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References
Collins RT. Cardiovascular disease in Williams syndrome. Curr Opin Pediatr 2018;30:609-615
Mavroudis C. Pediatric Cardiac Surgery. Wiley-Blackwell, 2013:601-612
Stamm C, Li J, Ho SY, et al. The aortic root in supravalvular aortic stenosis: the potential surgical relevance of morphologic findings. J Thorac Cardiovasc Surg 1997; 114:16–24
Braunstein PW Jr, Sade RM, Crawford FA Jr, et al. Repair of supravalvar aortic stenosis: cardiovascular morphometric and hemodynamic results. Ann Thorac Surg 1990; 50:700–707
Sharma BK, Fujiwara H, Hallman GL, et al. Supravalvar aortic stenosis: a 29-year review of surgical experience.
Ann Thorac Surg 1991;51:1031–1039
