Aortic supravalvular stenosis is a fixed kind of inborn left ventricular leakage parcel interference that occurs as a localized or a diffuse narrowing of the ascending aorta beyond the excellent edge of the sinuses of Valsalva. Severe left ventricular leakage parcel interference and coronary artery disease may too induce liberal dyspnea upon exertion, angina, and syncope in serious cases and may account for the morbidity in aortic supravalvular stenosis. Bacterial endocarditis can too induce mortality and morbidity in aortic supravalvular stenosis. The sporadic form of aortic supravalvular stenosis is the most common presentation. Patients may have associated peripheral pulmonary artery stenosis but show no other features of Williams syndrome.
Aortic supravalvular stenosis may happen periodically, as an expression of elastin arteriopathy, or as region of williams syndrome, a hereditary disorder with autosomal predominant heritage. Sudden death can happen in raw patients with aortic supravalvular stenosis. However, this appears to be comparatively uncommon whole. Sudden death is more common with Williams syndrome and aortic supravalvular stenosis with diffuse peripheral pulmonary artery stenosis. Sudden death can occur in untreated patients with aortic supravalvular stenosis. However, this appears to be relatively rare overall. Patients with aortic supravalvular stenosis usually become symptomatic during childhood, but aortic supravalvular stenosis is usually identified during infancy in cases associated with Williams syndrome.
The symptoms which are caused by supravalvar aorta constriction usually develop in childhood and do in small child time, however, some patients may develop symptoms in the second or third decade of life. Newborn infants with acute aortic stenosis present with signs of circulatory collapse, cyanosis, or congestive heart failure. Hypotension, tachycardia, respiratory distress, irritability, and poor peripheral perfusion are nonspecific signs of physiologic distress, RV hypertrophy will be present. Hepatomegaly may be present, as well peripheral edema and decreased peripheral pulses. Respiratory distress may be present. A condition known as Williams Synrome is associated with nearly half the cases of supravalvular aortic stenosis. Cardiac silhouette may be variably increased, and the ascending aorta may be asymmetrically dilated. The presence of both findings indicates hemodynamically significant aortic supravalvular stenosis.
A suitable diagnosis of aorta supravalvular constriction cannot be made, and the patients and their families can observe continuation care badly. Surgery is primary treatment for supravalvar aorta constriction. Catheter intervention in the form of transcatheter balloon angioplasty has not been found to be an effective treatment because the relief in gradient across aortic supravalvular stenosis is usually small and transient. Immediate surgical intervention is necessary, and may come in the form of balloon valvuloplasty, this, however, may result in complications relating to the small size and age of the patient. Open repair under direct vision is usually preferred. Percutaneous arterial stenting has been used to correct peripheral vascular stenoses, while surgical correction, either with patch augmentation or tube graft replacement, is favored for supravalvular aortic stenosis.