Left Ventricular Apical Aneurysm as a Consequence of Diffuse Type Congenital Nonfamilial Supravalvular Aortic Stenosis in a 30-Year-old Female

Abstract

SUMMARYCongenital nonfamilial supravalvular aortic stenosis (SVAS) is relatively rare, its diffuse type being the least common. We present a 30-year-old woman with diffuse SVAS complicated with left ventricular apical aneurysm. We believe that subtle left ventricular myocardial ischemia or infarction and long-lasting severe pressure overload to the apical chamber caused LV apical aneurysm in our case. Acquired LV apical aneurysm secondary to supravalvular aortic stenosis, in the absence of atherosclerotic coronary artery disease and hypertrophic obstructive cardiomyopathy, has not been described before. (Int Heart J 2005; 46: 153-159)

Supravalvular aortic stenosis in adult with anomalies of aortic arch vessels and aortic regurgitation

“…The supravalvular aortic stenosis (SAS) is the least common form of obstruction of the left ventricular outflow tract, representing 0.05% of all congenital heart disease [ 1 - 3 ] . It is characterized as a congenital obstruction of the ascending aorta, most commonly involving the sinotubular junction and may occur as a dysmorphia like an "hourglass" or as a diffuse hyperplasia.…”

Atypical initial presentation of Takayasu arteritis as isolated supra-valvular aortic stenosis

“…Mural thrombus in the left ventricular apical aneurysm may have been responsible for ischemic presentation in the patient. We believe that long-standing intra-ventricular pressure overload was caused by supra-valvular aortic stenosis, which might have resulted in left ventricular apical ischaemia in a quiet way and an ultimate aneurysmal change [ 8 ].…”