Aortic Stenosis in Children
What is aortic stenosis?
Aortic stenosis means that your child has a heart valve that is too narrow or is blocked.
The aortic valve is 1 of 4 heart valves that keep blood flowing through the heart.
The valves make sure blood flows in only one direction. The aortic valve keeps blood
flowing from the left ventricle to the aorta. Your child may be born with aortic stenosis
(congenital). Or it may develop later (acquired). It occurs more often in boys than
A normal aortic valve has 3 flaps (leaflets) that act as a one-way door. In aortic
stenosis, the valve doesn’t work as it should or has an abnormal number of leaflets
that don't work correctly. That makes it harder for the leaflets to open and let blood
flow from the left ventricle to the aorta.
Aortic stenosis may be mild, moderate, or severe. It depends on how much of the blood
is blocked. The condition may get worse over time. It may also occur with other heart
problems or conditions.
Moderate to severe aortic stenosis may affect the heart and blood vessels in these
- Over time the left ventricle becomes larger and can’t pump blood to the body very
- The aorta may also become larger.
- The coronary arteries that send blood to the heart muscle may not get enough blood.
What causes aortic stenosis?
A child can be born with aortic stenosis. This means the aortic valve didn’t form
as it should before birth. Sometimes this problem is caused by a genetic problem.
But most of the time, the cause for this isn’t known. In older children, aortic stenosis
may occur after an untreated strep infection.
What are the symptoms of aortic stenosis?
The symptoms of aortic stenosis vary, depending on how old your child is. They also
vary by how severe the blockage is. For example, a child with mild aortic stenosis
may have few symptoms. Or he or she may not have any symptoms. Symptoms may not show
up until adulthood. Or an infant may have trouble feeding and may not gain weight.
In severe (critical) aortic stenosis, infants are very ill.
More severe aortic stenosis may cause:
- Fatigue or tiredness
- Bluish discoloration around the lips or skin indicating low oxygen levels (cyanosis)
- Poor feeding
- Poor weight gain
- Dizziness or lightheadedness, especially with physical activity
- Fainting (syncope)
- Shortness of breath or rapid breathing
- Irregular heartbeats or feeling the heart beat (palpitations)
- Chest pain or pressure
The symptoms of aortic stenosis can be like other health conditions. Make sure your child
sees his or her healthcare provider for a diagnosis.
How is aortic stenosis diagnosed?
Your child's healthcare provider may have heard a heart murmur when listening to your
child’s chest with a stethoscope. A heart murmur is an abnormal sound as blood moves
through the heart. A heart murmur may mean that your child has a heart defect. Your
child’s symptoms are also part of figuring out the diagnosis.
Your child may need to see a pediatric cardiologist to confirm the diagnosis. This
is a doctor with special training to treat heart defects and other heart problems
in children. Your child may also have tests, such as:
- Chest X-ray. This gives an overall picture of your child's heart and lungs.
- Electrocardiogram (ECG). This test that measures the electrical activity of the heart.
- Echocardiogram (Echo). This test uses sound waves to give a moving picture of the heart and valves. This
is one of the best tests for aortic stenosis.
- Cardiac catheterization. This test shows details of the structures of the heart. Your child will have this
test while asleep. The doctor will put a thin, flexible tube (catheter) into one of
your child’s blood vessels. The doctor will slowly guide the catheter to the heart.
Contrast dye may be injected to let the cardiologist to see more detail.
- Exercise testing. This allows the doctor to examine the child's EKG during exercise.
- Pulse oximetry. This non invasive test measures oxygen levels in the blood through a clip placed
on a finger.
How is aortic stenosis treated?
Treatment will depend on your child’s symptoms, age, and general health. It will also
depend on how severe the condition is. If your child doesn’t have symptoms or if symptoms
are mild, his or her healthcare provider may just watch symptoms closely. This means
your child may often have office visits and tests.
The pediatric cardiologist and a cardiothoracic surgeon will figure out whether your
child needs an aortic valve procedure. The procedures include:
- Balloon aortic valvuloplasty. This is done with cardiac catheterization using a catheter with a deflated balloon
in the tip. The catheter is put into a blood vessel. It is moved to the narrowed
valve, and the balloon is inflated to open the valve. Many providers prefer this procedure.
- Surgical aortic valvotomy. This is surgery to remove scar tissue from the aortic valve leaflets. This lets the
leaflets to open as they should.
- Aortic valve replacement. This is surgery to replace the aortic valve with a new valve. Replacement valves are
either artificial or from donor organs or animals.
- Pulmonary autograft (Ross procedure). This is surgery to replace the aortic valve and part of the aorta. Your child's
own pulmonary valve and part of the pulmonary artery are used to replace the damaged
aortic valve. A pulmonary valve and part of the pulmonary artery from a donor organ
are used to replace the transplanted valve and artery. Many surgeons prefer this method
because it continues to work well as a child grows.
Before the procedures you might expect the following:
- An infant who has critical aortic stenosis will be in an intensive care unit (ICU).
He or she may need emergency repair of the valve. Infants who aren’t as sick have
the procedure planned.
- A child with severe aortic stenosis may not be able to take part in sports. This is
especially true for sports that have intense or long periods of activity.
What are the complications of aortic stenosis?
Complications of moderate to severe aortic stenosis include:
- Bulging or weakening (aneurysm) of the aorta
- Tear (dissection) of the aorta
- Infection of the lining of the heart, valves, or blood vessels (infective endocarditis)
- Heart is not able to pump as it should (heart failure)
Talk with your child’s healthcare provider about his or her risk for these problems.
Can aortic stenosis be prevented?
Congenital aortic stenosis can’t be prevented. But all newborns are screened for congenital
heart disease with pulse oximetry.
This is a simple, painless test to check the amount of oxygen in the blood. It’s
done by placing a small probe on the infant’s arm and leg. If the oxygen level is
low, it may mean there is a heart defect. More testing and treatment will be done
if a problem is found.
What is living with aortic stenosis like after a procedure?
Most children who have had an aortic valve repair or replacement live active, healthy
lives. Your child’s activity levels, appetite, and growth usually return to normal.
Your child should get regular follow-up care with a cardiologist throughout his or
her life. Your child may also need:
- Regular blood pressure checks and management of high blood pressure
- Regular dental care. This is to prevent infections that may lead to a heart infection (endocarditis).
- Limited physical activity. Talk with your child’s healthcare provider about safe activities for your child.
- Antibiotics before procedures. This includes dental work. This depends on whether your child has had a valve repair
- Blood thinners (anticoagulants). These prevent blood clots from forming on a mechanical valve. Blood tests to check
the blood thinners are also done.
- Possible repeat valve repair or replacement
Talk with your healthcare provider about what sports activities are appropriate for
When should I call my child’s healthcare provider?
Contact your child’s healthcare provider if you notice:
- Symptoms such as chest pain or trouble breathing that get worse
- Dizziness or tiredness with physical activity
If you child has had a procedure, make sure to follow all instructions from the surgeon.
And make sure to keep all follow-up appointments with your child’s cardiologist and
Key points about aortic stenosis
- Aortic stenosis means that a valve in your child’s heart is too narrow or is blocked.
This condition can be mild, moderate, or severe.
- Common symptoms are tiredness, poor feeding and weight gain, dizziness, shortness
of breath, chest pain or discomfort, and a fast heartbeat.
- A pediatric cardiologist usually diagnoses and manages aortic stenosis.
- Several procedures are available to repair or replace the aortic valve.
- First degree relatives of your child should discuss the need for screening for aortic
stenosis with their healthcare provider.
- A child with aortic stenosis should be checked often. Follow-up care is needed throughout
his or her life.
Tips to help you get the most from a visit to your child’s healthcare provider:
- Know the reason for the visit and what you want to happen.
- Before your visit, write down questions you want answered.
- At the visit, write down the name of a new diagnosis, and any new medicines, treatments,
or tests. Also write down any new instructions your provider gives you for your child.
- Know why a new medicine or treatment is prescribed and how it will help your child.
Also know what the side effects are.
- Ask if your child’s condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if your child does not take the medicine or have the test or procedure.
- If your child has a follow-up appointment, write down the date, time, and purpose
for that visit.
- Know how you can contact your child’s provider after office hours. This is important
if your child becomes ill and you have questions or need advice.