stenosis is a heart defect that may be congenital (present at birth) or
acquired (develop later in life). If the problem is congenital, then
something occurred during the first eight weeks of pregnancy to affect
the development of the aortic valve.
The aortic valve is found between the left ventricle and the aorta. It
has three leaflets that function like a one-way door, allowing blood to
flow forward into the aorta, but not backward into the left ventricle.
Aortic stenosis is the inability of the aortic valve to open completely.
With aortic stenosis, problems with the aortic valve make it harder for
the leaflets to open and permit blood to flow forward from the left
ventricle to the aorta. In children, these problems can include a valve
Only has two leaflets instead of three (bicuspid aortic valve).
Has leaflets that are partially fused together.
Has thick leaflets that do not open all the way.
Becomes damaged by rheumatic fever or bacterial endocarditis.
Area above or below the valve is narrowed (supravalvar or
Aortic stenosis may be present in varying degrees, classified according
to how much obstruction to blood flow is present. A child with severe
aortic stenosis will be quite ill, with major symptoms noted early in
life. A child with mild aortic stenosis may have few symptoms, or
perhaps none until later in adulthood. The degree of obstruction can
become worse with time.
Congenital aortic stenosis occurs in four to six percent of all children
with congenital heart disease. Relatively few children are symptomatic
in infancy, but the incidence of problems increases sharply in
Congenital aortic stenosis occurs three times more often in boys than in
Congenital aortic stenosis occurs due to improper development of the
aortic valve in the first 8 weeks of fetal growth. It can be caused by a
number of factors, though, most of the time, this heart defect occurs
sporadically (by chance), with no apparent reason for its development.
Some congenital heart defects may have a genetic link, either occurring
due to a defect in a gene, a chromosome abnormality, or environmental
exposure, causing heart problems to occur more often in certain
Acquired aortic stenosis may occur after a strep infection that
progresses to rheumatic fever.
Mild aortic stenosis may not cause any symptoms. Several problems may
occur, however, when aortic stenosis is moderate to severe, including
The left ventricle has to work harder to try to move blood
through the tight aortic valve. Eventually, the left ventricle
is no longer able to handle the extra workload, and it fails to
pump blood to the body efficiently.
There is a higher than average chance that the aorta may become
dilated (enlarged). This can increase the risk of an aneurysm or
dissection of the aorta.
There is a higher than average chance of developing an infection
in the lining of the heart or aorta known as bacterial
The coronary arteries, which supply oxygen-rich (red) blood to
the heart muscle, may not receive enough blood to meet the
demands of the heart.
The following are the most common symptoms of aortic stenosis. However,
each child may experience symptoms differently. Symptoms may include:
Dizziness with exertion
Shortness of breath
Irregular heartbeats or palpitations
The symptoms of aortic stenosis may resemble other medical conditions or
heart problems. Always consult your child's physician for a diagnosis.
Your child's physician may have heard a heart murmur during a physical
examination, and referred your child to a pediatric cardiologist for a
diagnosis. A heart murmur is simply a noise caused by the turbulence of
blood flowing through the obstruction from the right ventricle to the
pulmonary artery. Symptoms your child exhibits will also help with the
A pediatric cardiologist specializes in the diagnosis and medical
management of congenital heart defects, as well as heart problems that
may develop later in childhood. The cardiologist will perform a physical
examination, listening to your child's heart and lungs, and make other
observations that help in the diagnosis. The location within the chest
that the murmur is heard best, as well as the loudness and quality of
the murmur (harsh, blowing, etc.) will give the cardiologist an initial
idea of which heart problem your child may have. Diagnostic testing for
congenital heart disease varies by the child's age, clinical condition,
and institutional preferences. Some tests that may be recommended
include the following:
Chest X-ray. A diagnostic test which uses X-ray beams to produce
images of internal tissues, bones, and organs onto film.
Electrocardiogram (ECG or EKG). A test that records the
electrical activity of the heart, shows abnormal rhythms
(arrhythmias or dysrhythmias), and detects heart muscle stress.
Echocardiogram (echo). A procedure that evaluates the structure
and function of the heart by using sound waves recorded on an
electronic sensor that produce a moving picture of the heart and
Exercise electrocardiogram (ECG or EKG). An exercise EKG is done
to assess the heart's response to stress or exercise. The EKG is
monitored while your child is exercising on a treadmill or
stationary bike. An EKG measures the electrical activity of your
Cardiac MRI. A diagnostic procedure that uses a combination of
large magnets, radiofrequencies, and a computer to produce
detailed images of the heart.
Cardiac catheterization. A cardiac catheterization is an
invasive procedure that gives very detailed information about
the structures inside the heart. Under sedation, a small, thin,
flexible tube (catheter) is inserted into a blood vessel in the
groin, and guided to the inside of the heart. Blood pressure and
oxygen measurements are taken in the four chambers of the heart,
as well as the pulmonary artery and aorta. Contrast dye is also
injected to more clearly visualize the structures inside the
Specific treatment for aortic stenosis will be determined by your
child's physician based on:
Your child's age, overall health, and medical history
Extent of the disease
Your child's tolerance for specific medications, procedures, or
Expectations for the course of the disease
Your opinion or preference
Aortic stenosis is treated with repair of the obstructed valve. Several
options are currently available.
Some infants will be very sick, require care in the intensive care unit
(ICU) prior to the procedure, and could possibly even need emergency
repair of the aortic stenosis. Others, who are exhibiting few symptoms,
will have the repair scheduled on a less urgent basis.
Activity may be limited in children who have moderate aortic stenosis
prior to repair. For instance, competitive sports that require endurance
may be restricted.
Repair options include the following:
Balloon dilation. A cardiac catheterization procedure, a small,
flexible tube (catheter) is inserted into a blood vessel in the
groin, and guided to the inside of the heart. The tube has a
deflated balloon in the tip. When the tube is placed in the
narrowed valve, the balloon is inflated to stretch the area
open. Children who have undergone balloon dilation will need to
follow antibiotic prophylaxis for a specific period of time
after discharge from the hospital.
Valvotomy. Surgical release of scar tissue within the aortic
valve leaflets that are preventing the valve leaflets from
Aortic valve replacement. The aortic valve is replaced with a
new valve. Replacement valves fall into two categories: Tissue
(biological) valves, which are made from animal tissues and
valves. The valve leaflets are soft and thin. On the other hand,
mechanical valves, which are made from carbon fibers, have solid
valve leaflets. Children who have undergone a valve replacement
will need to follow antibiotic prophylaxis throughout their
lifetime. Patients who have received a mechanical valve will
need life-long treatment with coumadin, a medication that thins
the blood to prevent catastrophic clots from forming on the
valve leaflets themselves.
Aortic homograft. A section of aorta from a human donor with its
aortic valve intact is used to replace the aortic valve and a
section of the ascending aorta. This procedure is seldom
performed due to the complexity of the operation and the rapid
failure of the aortic homograft itself.
Pulmonary autograft (Ross procedure). A section of the child's
own pulmonary artery with the pulmonary valve intact is used to
replace the aortic valve and a section of the ascending aorta. A
section of pulmonary artery from another human donor with its
valve intact is used to replace the transferred pulmonary
After surgery, your child will go to the intensive care unit (ICU).
While your child is in the ICU, special equipment will be used to help
him or her recover from surgery, and may include the following:
Ventilator. A machine that helps your child breathe while he or
she is under anesthesia during the operation. A small, plastic
tube is guided into the windpipe and attached to the ventilator,
which breathes for your child while he or she is too sleepy to
breathe effectively on his or her own. Many children remain on
the ventilator for a while after surgery so they can rest.
Intravenous (IV) catheters. Small, plastic tubes inserted
through the skin into blood vessels to provide IV fluids and
important medicines that help your child recover from the
Arterial line. A specialized IV placed in the wrist, or other
area of the body where a pulse can be felt, that measures blood
pressure continuously during surgery and while your child is in
Nasogastric (NG) tube. A small, flexible tube that keeps the
stomach drained of acid and gas bubbles that may build up during
Urinary catheter. A small, flexible tube that allows urine to
drain out of the bladder and accurately measures how much urine
the body makes, which helps determine how well the heart is
functioning. After surgery, the heart will be a little weaker
than it was before, and, therefore, the body may start to hold
onto fluid, causing swelling and puffiness. Diuretics may be
given to help the kidneys remove excess fluid from the body.
Chest tube. A drainage tube may be inserted to keep the chest
free of blood that would otherwise accumulate after the incision
is closed. Bleeding may occur for several hours, or even several
days after surgery.
Heart monitor. A machine that constantly displays a picture of
your child's heart rhythm, and monitors heart rate, arterial
blood pressure, and other values.
Your child may need other equipment not mentioned here to provide
support while in the ICU, or afterwards. The hospital staff will explain
all of the necessary equipment to you.
Your child will be kept as comfortable as possible with several
different medications; some of which relieve pain and some of which
relieve anxiety. The staff will also be asking for your input as to how
best to soothe and comfort your child.
After discharge from the ICU, your child will recuperate on another
hospital unit for a few days before going home. You will learn how to
care for your child at home before your child is discharged. Your child
may need to take medications for a while, and these will be explained to
you. The staff will give you instructions regarding medications,
activity limitations, and follow-up appointments before your child is
Most children who have had an aortic stenosis surgical repair will live
healthy lives. Activity levels, appetite, and growth should eventually
return to normal.
As the child grows, a valve that was ballooned may once again become
narrowed. If this happens, a second balloon procedure or operation may
be necessary to repair aortic stenosis. Sometimes the aortic tissue
itself may be abnormal, which might lead to complications in the teen or
adult years. Regular follow-up care at a specialized cardiac center
should continue throughout life.
Your child's cardiologist may recommend that antibiotics be given to
prevent bacterial endocarditis after discharge from the hospital.
Individuals who had a mechanical valve replacement may need to take
anticoagulants (blood thinners) to prevent blood clots from forming on
the artificial valve surfaces. Regular monitoring of the blood’s
clotting status is very important in maintaining the most appropriate
dose of anticoagulants.
Initial valve replacement is often performed using a tissue valve to
avoid the need for anticoagulation, especially for females of
childbearing age. Anticoagulation during pregnancy is very difficult to
manage, and requires special treatment.
Repeat valve replacement is not uncommon during the lifespan. In
addition, blood pressure should be closely monitored and managed.
Consult your child's physician regarding the specific outlook for your
For more information, call 1.866.CALL.MLH.