Is Supravalvular aortic stenosis serious?

If SVAS is not treated, the aortic narrowing can lead to shortness of breath, chest pain, and ultimately heart failure. The severity of SVAS varies considerably, even among family members. Some affected individuals die in infancy, while others never experience symptoms of the disorder.

How can you tell the difference between aortic stenosis and pulmonary stenosis?

Unlike the aortic stenosis murmur, a pulmonic stenosis murmur does not radiate, and the crescendo component lengthens as stenosis progresses. The murmur grows louder immediately with Valsalva release and with inspiration; the patient may need to be standing for this effect to be heard.

Which syndrome is associated with Supravalvular aortic stenosis?

Supravalvular aortic stenosis (SVAS) can either be isolated or can occur as part of Williams syndrome.

Can babies have aortic stenosis?

Critical aortic stenosis is a term used in newborns with very severe narrowing , requiring treatment soon after birth. Over time, if the problem is not treated, this overwork causes a thickening of the heart muscle called ventricular hypertrophy.

How is Supravalvular aortic stenosis treated?

Surgery is the primary treatment for supravalvar aortic stenosis (SVAS). Children and adolescents with catheter peak-to-peak (or Doppler mean) gradient of 50 mm Hg or more should have surgical intervention. The choice of procedures in these patients is similar to that indicated for valvar aortic stenosis.

How common is pulmonary stenosis in babies?

Pulmonary stenosis is relatively common and accounts for about 10% of heart defects diagnosed during childhood. It can occur in children with otherwise normal hearts or along with other congenital heart defects such as atrial septal defect or Tetralogy of Fallot.

Can a child outgrow pulmonary stenosis?

Because pulmonary stenosis can be a lifelong condition, kids who have the defect will need to see a cardiologist (a doctor who specializes in treating heart problems) regularly to make sure the narrowing isn’t getting worse.

What is Supravalvular pulmonary stenosis?

In supravalvar pulmonary stenosis, the narrowing is in the artery just beyond the pulmonary valve. Patches are sewn into this artery to enlarge its diameter and relieve the narrowing.

What is pulmonary stenosis in babies?

Pulmonary stenosis is a birth defect of the heart (congenital). It can happen when the pulmonary valve doesn’t grow as it should in a baby during the first 8 weeks of pregnancy. The pulmonary valve connects the right ventricle to the pulmonary artery. It normally has 3 flaps (leaflets) that work like a 1-way door.

How common is Supravalvular aortic stenosis?

SVAS accounts for less than 0.05% of all congenital heart defects. SVAS accounts for 8% to 14% of all congenital aortic stenosis. The sporadic form of SVAS is a more common than the autosomal dominant form.

What are the symptoms of aortic valve stenosis?

Symptoms of severe aortic valve stenosis include: chest pain as the heart strains to pump enough blood through the compromised valve. feeling tired after exertion, as when you exercise or move. feeling short of breath, especially after exertion.

Can aortic stenosis be reversed?

However, there are no medications that can reverse aortic stenosis. The only cure is replacing the diseased valve. Usually, heart valve replacement requires open-heart surgery. During this procedure, patients are connected to a heart-lung bypass machine, which does the work of the heart while their heart is stopped.

What is the prognosis of severe aortic stenosis (as)?

If untreated: Eventually if it goes untreated. Once patients become symptomatic from severe aortic stenosis (chest pain, shortness of breath, passing out) then the 2 year survival is only about 50%. Therefore patients with aortic stenosis should see their cardiologist regularly.

Is aortic stenosis a progressive disease?

Typically, aortic stenosis (AS) is a progressive disease. The cross-section of a normal aortic valve is about the size of nickel (3 to 4 cm2). In aortic stenosis, this cross-section becomes smaller. As aortic stenosis progresses, the left ventricle, the main pumping chamber of the heart, must compensate by increasing the pressure it must