Aortic supravalvular stenosis

Below you will find more information about Aortic supravalvular stenosis from Medigest. If you believe that you are suffering from any of the symptoms of Aortic supravalvular stenosis it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition. There are medical conditions that carry similar symptoms associated with Aortic supravalvular stenosis and therefore the information provided by Medigest is offered as a guideline only and should never be used in preference to seeking professional medical advice. The information relating to Aortic supravalvular stenosis comes from a third party source and Medigest will not be held liable for any inaccuracies relating to the information shown.

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.

Diagnosis

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

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.

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.

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