Abstract

Supravalvular aortic stenosis (SAS) is a heart defect that develops before birth. This defect is a narrowing (stenosis) of the aorta and the condition is described as supravalvular because the section of the aorta that is narrowed is located just above the aortic valve. SAS represents 8% to 14% of all congenital aortic stenosis. SAS has been classified in various ways: hourglass, membranous, and hypoplasia of aortic arch or more commonly in discrete or diffuse. Hourglass or discrete type SAS is the most common [1,2]. Some people with SAS also have defects in other blood vessels, most commonly stenosis of the pulmonary arteries. An abnormal heart sound during a heartbeat (heart murmur) can often be heard during a chest exam. If SAS is not treated, the aortic narrowing can lead to shortness of breath, chest pain, and ultimately heart failure. The severity of SAS varies considerably, even among family members. Some affected individuals need treatment in infancy, while others never experience symptoms of the disorder.

Keywords:Aorta; Supravalvar; Hypertrophy