Mechanism of elevated innominate artery pressures in supravalvular aortic stenosis.

Late sequela of aortotomy in infancy: supravalvular aortic stenosis and aneurysmal changes of epiaortic branches.

Figure 1: Preoperative computed tomography showed hourglass type of supravalvular aortic stenosis and aneurysmal changes of the innominate and the left subclavian artery. When she underwent ventricular septal defect repair at the age of 4 months, transverse aortotomy was made for evaluating a bicuspid aortic valve. Probably the transverse aortotomy was closed by a continuous suture, which, by d...

Elastin insufficiency predisposes to elevated pulmonary circulatory pressures through changes in elastic artery structure.

Elastin is a major structural component of large elastic arteries and a principal determinant of arterial biomechanical properties. Elastin loss-of-function mutations in humans have been linked to the autosomal-dominant disease supravalvular aortic stenosis, which is characterized by stenotic lesions in both the systemic and pulmonary circulations. To better understand how elastin insufficiency...

An explanation of asymmetric upper extremity blood pressures in supravalvular aortic stenosis: the Coanda effect.

Left ventricular apical aneurysm as a consequence of diffuse type congenital nonfamilial supravalvular aortic stenosis in a 30-year-old female.

Congenital 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....

Non ST-Elevation Myocardial Infarction in a Patient with Supravalvular Aortic Stenosis. Role of Multi-Modality Imaging.

Dear Editor, Supravalvular aortic stenosis (SVAS) is a congenital narrowing of the ascending aorta above the level of sinus of Valsalva. It is most often commonly associated with William’s syndrome although it can arise without syndromic associations as well.1,2 Left main coronary artery (LMCA) occlusion or stenosis has been seen to be associated with the condition, and can be a cause of sudden...