Pediatric aortic stenosis

What is pediatric aortic stenosis?

Pediatric aortic stenosis is a heart condition that happens when blood flow is blocked because the aortic valve is too small or narrow. The aortic valve is made of three tiny flaps of skin (leaflets) that open and close with each heartbeat, controlling blood flow like a one-way street.

With aortic stenosis, the flaps stick together or become too thick, which narrows the valve and prevents it from opening and closing the right way. This causes the heart to work harder, weakening it over time.

What are the signs and symptoms of pediatric aortic stenosis?

The signs and symptoms of aortic stenosis will differ depending on the severity or level of blockage. In mild to moderate cases, children will usually have a heart murmur and no other signs.

In severe cases, symptoms may include:

• Fatigue

• Dizziness

• Fainting spells, especially with physical activities like sports

• Shortness of breath or difficulty breathing

• Signs of heart failure, like visible swelling of the legs, face, or tummy and a sudden change in temperature or skin

How is pediatric aortic stenosis diagnosed?

Diagnosing pediatric aortic stenosis diagnosis usually starts with doctors hearing a heart murmur during a routine exam. A heart murmur is a type of sound the heart makes when there is a valve problem or blood flow is being blocked. This can be detected while the baby is still in the womb with a routine prenatal ultrasound and in older children during a pediatric wellness exam with a stethoscope.

If your physician hears a heart murmur, they may refer you to us for further testing to confirm the diagnosis.

Echocardiogram - At Children’s Health, the gold standard for diagnosing aortic stenosis in unborn babies and older children is an echocardiogram, or “echo”. This is an ultrasound imaging test that measures the aortic valve and heart, helping us see how a child’s heart looks and works.

It can be performed as early as 16 weeks into pregnancy. This means aortic stenosis can be identified before a baby is born, which allows us to closely monitor the condition and determine if treatment is necessary at birth.

What causes pediatric aortic stenosis?

Aortic stenosis happens when the aortic valve doesn’t develop properly during pregnancy. This irregularity can be genetic but most of the time it develops by chance, for no known reason.

How is pediatric aortic stenosis treated?

Treatment for aortic stenosis will depend on:

• The amount of valve narrowing (stenosis)

• How much blood flow is blocked

• The size of the aortic valve

• The severity of symptoms

• The age of your child

• Your preferences

In mild cases, treatment isn’t usually necessary. But the condition can get worse over time, which means your child might require treatment later in life. Our pediatric cardiologists will work with your child’s pediatrician to watch their condition closely for any changes that may require treatment.

Moderate to severe cases almost always require treatment to repair the aortic valve. At Children’s Health, our skilled cardiologists are experts in performing treatments for aortic stenosis.

• Balloon valvuloplasty - This is done with a technique called cardiac catheterization. A thin tube (catheter) with a deflated balloon on the end is placed into the aortic valve. The balloon is then slowly inflated to open the valve.

• Aortic valve replacement (AVR) - This is a type of surgery to replace the damaged valve with a new one. Replacement valves can be mechanical or come from a donor (person or animal).

• Ross procedure (pulmonary autograft) - This procedure replaces the aortic valve and part of the aorta by using a different section of your child’s heart (their pulmonary valve and part of their pulmonary artery.)