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Aortic supravalvular stenosis

Aortic supravalvular stenosis Causes

The precise etiology of SVAS is not known. Its high association with Williams syndrome, in which an elastin gene mutation is present, implies that defective connective tissue formation contributes to its pathology.

Aortic supravalvular stenosis Definition

Supravalvular aortic stenosis (SVAS) is a fixed form of congenital left ventricular outflow tract (LVOT) obstruction that arises as a localized or a diffuse narrowing of the ascending aorta beyond the superior margin of the sinuses of Valsalva. It is responsible for less than 7% of all fixed forms of congenital LVOT obstructive lesions. SVAS may arise sporadically, as a manifestation of elastin arteriopathy, or as part of Williams syndrome (also known as Williams-Beuren syndrome), a genetic disorder with autosomal dominant inheritance.

Aortic supravalvular stenosis Diagnosis

No specific laboratory blood tests are needed to establish the diagnosis of supravalvar aortic stenosis

Aortic supravalvular stenosis Symptoms and Signs

When symptomatic, aortic stenosis can result to dizziness, syncope, angina and congestive heart failure. More symptoms may indicate a worse prognosis. Treatment needs replacement of the diseased valve with an artificial heart valve.

Aortic supravalvular stenosis Treatment

In adults, aortic stenosis typically requires aortic valve replacement if medical management does not successfully control symptoms. According to a prospective, single-center, nonrandomized study of 25 patients, percutaneous implantation of an aortic valve prosthesis in high risk patients with aortic stenosis leads to marked hemodynamic and clinical improvement when successfully completed. For infants and children, balloon valvuloplasty, where a balloon is inflated to stretch the valve and allow greater flow, may also prove to be effective.

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