Fetal Aortic Valve Stenosis

What are the signs and symptoms of aortic valve stenosis?

The signs and symptoms of aortic valve stenosis differ by age and degree of stenosis.

Fetal aortic valve stenosis

If the stenosis is severe, aortic valve stenosis may be detected before a baby is born. A fetus with critical aortic stenosis may develop signs of heart failure, such as fluid around the heart called hydrops. In the case of severe aortic stenosis, the left ventricle may become very thickened (hypertrophied) and the ventricle may function poorly. Sometimes the left ventricle may decrease in size and evolve into hypoplastic left heart syndrome.

Aortic valve stenosis in infants

After birth, babies with severe aortic stenosis often have fast and labored breathing and difficulty feeding. Infants may also be irritable and weak. They can have signs of low blood flow to the body, which include pale skin color and cool extremities with poor pulses. If not treated, severe aortic stenosis can cause a baby to go into shock because the body organs are not receiving proper blood flow.

Aortic valve stenosis in children and adolescents

A child or adolescent with mild aortic stenosis may have no symptoms, whereas a child or adolescent with severe aortic stenosis may have decreased endurance and become winded easily with exercise. They may also experience chest pain or pass out with exercise.

What tests are used to diagnose aortic valve stenosis?

If we suspect that is there is something wrong with your baby’s heart while they are in the womb, we will perform a test called a fetal echocardiogram. A fetal echocardiogram is an ultrasound of the baby’s heart. This ultrasound provides information about your baby’s heart and helps us decide how best to manage your baby’s condition.

Here in our Maternal Fetal Medicine Program, we can perform a fetal echocardiogram as early as 14 weeks gestation. A fetal echocardiogram is safe for mom and baby and does not cause any pain or negative effects for the baby.

After birth, we diagnose aortic valve stenosis using an echocardiogram.

How is aortic valve stenosis treated?

The treatment of aortic stenosis depends on the severity of the stenosis. For mild stenosis, no treatment may be needed. For severe stenosis, a catheterization procedure called a balloon aortic valvuloplasty is indicated. If the stenosis has caused the left ventricle to become too small, then surgery is needed.

Balloon aortic valvuloplasty

A balloon aortic valvuloplasty is a cardiac catheterization procedure. A highly specialized cardiologist called an interventional cardiologist will perform this procedure. During this procedure, a thin plastic tube (a catheter) is placed through the vessels in the leg and the catheter is directed into the heart across the aortic valve using a thin wire. The balloon attached to the wire is inflated across the aortic valve. This helps to stretch the valve so that valve can open and the blood can flow to the body without obstruction. The procedure takes several hours, and your baby usually recovers from the procedure the following day. A small bandage is placed on your baby’s leg where the catheter was inserted.

Learn more about cardiac catheterization procedures.

Fetal aortic valve stenosis

We follow babies with severe aortic valve stenosis very carefully by regular fetal echocardiograms to monitor the left ventricular function and watch for any signs of heart failure. We prefer to allow your baby to get as close to term (40 weeks) as possible before delivery to optimize success of the procedure. Even if the stenosis is severe, as long as your baby is growing and the left ventricle is pumping well and there are no signs of fluid around the heart (hydrops), then we still try to allow your baby to get to term for delivery if possible. We aim to deliver your baby by induction of labor at 39 weeks gestation to ensure an in-hospital delivery.

In some cases when the baby’s growth of the left ventricle appears to be hindered by the aortic stenosis, we may offer to do the balloon aortic valvuloplasty to open the valve while your baby is in the womb. This is a highly specialized procedure performed only in a select few centers around the country. Sometimes the balloon procedure before and after birth is not enough to open the valve. Your baby may need surgery to further open the aortic valve or to repair the valve if your baby develops significant insufficiency of the valve after the balloon procedure.

Surgery may be needed as a primary treatment if there are associated lesions such as a ventricular septal defect or aortic coarctation. Surgery may also be needed if there is significant dilation or narrowing of the aorta above the valve or narrowing below the valve. For this reason, we ensure that your family has also met with our surgical team early in the process.

Pediatric aortic valve stenosis

For severe aortic valve stenosis, your baby will most likely need a balloon aortic valvuloplasty to open the valve and improve blood flow to the body.

The catheterization team will be prepared for your baby’s arrival and ready to perform the balloon aortic valvuloplasty if your baby needs the procedure. Your baby will have an echocardiogram immediately after birth. Your baby will be closely monitored before and after the procedure in the cardiac intensive care unit.

Why choose us to treat aortic valve stenosis?

Here at Children’s Colorado, we take a team approach to ensure that your baby and family receive the specialized care that you need. Our Maternal Fetal Medicine team includes highly trained fetal cardiologists to oversee your baby’s care before birth and to ensure a safe delivery. In the case of a fetal diagnosis, experts in our Colorado Fetal Care Center can care for both mom and baby under the same roof, with effortless access to any pediatric specialists and subspecialists your child may need immediately following delivery. After birth, our pediatric cardiology team will ensure your child receives a seamless transition to care in our nationally ranked Heart Institute. We offer the most comprehensive treatment for congenital heart disease in the region, with specialized programs for cardiac catheterization, cardiac surgery, electrophysiology, adult congenital heart disease, wellness and more.

With pediatric cardiology quality and patient safety outcomes among the best in the nation, we’re the best choice for your child.

Helpful resources

• The American Heart Association is dedicated to fighting heart disease and stroke through awareness, research and education.
• The Fetal Health Foundation supports families who have received a fetal syndrome diagnosis, funds research, increases awareness and provides helpful information on fetal syndromes.
• The Pediatric Congenital Heart Association provides congenital heart disease education, support, research and awareness.