Pulmonary stenosis (also called pulmonic stenosis) is when the
pulmonary valve (the valve between the right ventricle and the pulmonary artery) is
too small, narrow, or stiff.
Symptoms of pulmonary stenosis depend on how small the narrowing of the pulmonary
valve is. If symptoms are mild, pulmonary stenosis may never require any treatment.
But kids with more severe pulmonary stenosis will need a procedure to fix the pulmonary
valve so blood can flow properly through the body.
What Happens in Pulmonary Stenosis?
The aortic valve and pulmonary valve control the flow of blood as it leaves the heart and keep it flowing forward. They
open to let blood move ahead, then quickly close to keep it from flowing backward.
The pulmonary valve lets the blood flow forward to the lungs. It opens to let blood
move ahead, then quickly closes to keep blood from flowing backward.
In pulmonary stenosis (pul-muh-NAIR-ee stuh-NO-sis), the pulmonary valve is too
small, too narrow, and can't open all the way. This causes the right ventricle to
pump harder to send blood out to the lungs. Over time, this can cause thickening of
the right ventricle and strain the heart.
What Are the Signs and Symptoms of Pulmonary Stenosis?
Many people with pulmonary stenosis have no symptoms. Others have mild symptoms
that usually don't become bothersome. In most cases of pulmonic stenosis, the doctor
will hear a heart murmur.
Symptoms of severe pulmonary stenosis can include:
in newborns, a bluish tint to the skin (called cyanosis) caused by blood that
is low in oxygen
being very tired
poor weight gain
shortness of breath
palpitations (sensation of rapid or irregular heartbeat)
a swollen abdomen
What Causes Pulmonary Stenosis?
In children, pulmonary stenosis happens when a baby's heart doesn't develop the
way it should during pregnancy. Doctors don't know why this happens, but it isn't
caused by anything a mother did or didn't do during her pregnancy, so could not have
Who Gets Pulmonary Stenosis?
Most cases of pulmonary stenosis are congenital, meaning a baby is born with it.
How Is Pulmonary Stenosis Diagnosed?
Doctors can often identify pulmonary stenosis before birth. This lets babies with
severe problems be treated right away.
A fetal echocardiogram (also called a fetal echo) is a type of
test that can help diagnose heart defects. A fetal echo uses sound waves to create
a moving picture of the heart. This helps doctors see how the baby's heart looks and
works while still in the mother's womb.
If the problem wasn't found before birth, infants and older kids who have a suspected
heart problem get an echocardiogram. Less commonly, a heart
catheterization may be done if a diagnosis isn't clear. In a catheterization,
a doctor inserts a catheter (a thin plastic, flexible tube) into an artery and vein
that lead to the heart.
How Is Pulmonary Stenosis Treated?
In some cases, pulmonary stenosis doesn't need to be treated. Medicines sometimes
can treat symptoms. In severe cases, though, the pulmonary valve will need to be fixed
Many types of procedures can repair or replace the pulmonary valve. Most severe
cases of pulmonic stenosis can be treated with a balloon valvuloplasty
during heart catheterization. With this procedure, a doctor threads an unopened balloon
through the pulmonary valve and inflates it to open the valve.
Valve replacement involves using an artificial valve or a valve
from a donor.
To decide what treatment to use, doctors consider:
the location of the narrowing and the amount of narrowing
the child's age and size
how well the other valves in the heart are working
whether the child has had previous heart surgery
whether the child has other medical conditions
A challenge for some kids with pulmonary stenosis is that it can come back again
after treatment. This can happen for different reasons, including scar tissue that
forms after a procedure or a valve replacement that doesn't grow as kids get bigger.
So some kids might need several procedures to keep the valve healthy.
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.
Many children won't need specific medical treatment, and those who do usually can
enjoy most regular activities after their recovery. Kids and teens with moderate or
severe pulmonary stenosis should talk with their cardiologist before playing competitive
sports or being very physically active.