Pulmonary Vein Stenosis

What is Pulmonary vein stenosis (PVS)

Pulmonary vein stenosis (PVS) is when 1 or more of the veins that carry blood from the lungs to the heart become too narrow. This makes it harder for blood to flow the way it should.

Why does it happen?

Doctors are not always sure why it starts.

Sometimes babies are born with it.
Other times, it can happen after heart surgery or because of other lung or heart problems.

How common is PVS?

PVS is rare. It happens in about 1 out of every 2,000 babies.

Are there different types?

Yes!

Some kids have it in just 1 vein.
Other kids have it in more than 1 vein.
It can also be part of other heart problems.

What are the symptoms?
Fast or hard breathing

Tiredness
Poor feeding
Slow weight gain
Pale or bluish skin
Coughing more than expected
Trouble breathing

How do doctors find it?

Doctors use special pictures of the heart and lungs, like:

Echocardiograms (heart ultrasound)
CT scans
Heart catheterization (A tiny tube goes into the heart to check blood flow.)
Lung Perfusion Scan (taking pictures of the amount of blood going to each lung)
MRI scans

Can it be part of other conditions?

Yes.

Kids with PVS may also:

have lung disease.
have heart defects.
be born early.

What do parents usually ask?

Will my child need surgery?
Can this be fixed?
Will my child grow up healthy?

We are here to answer all your questions. We support your family every step of the way.

Treatment at Children’s Mercy

How often do we treat this?

Children’s Mercy is one of the few places in the country that sees and treats many children with PVS each year. Our team has special training and experience with this rare condition.

What are the treatment options?

Balloon angioplasty: A small balloon opens the narrow vein.
Stents: Tiny tubes help keep the vein open.
Surgery: Sometimes needed to fix the vein.
Medicine: Helps slow down the narrowing.

Where does treatment happen?

Most treatments happen in the hospital. Some follow-up care can be done at home or in our outpatient clinics.

What can families expect?
A team of experts will meet with you.

We will explain the plan and answer your questions.
Your child may need more than one treatment over time.

Who will be on your child’s care team?
Pediatric cardiologist (heart doctor)

Heart surgeon
Nurse practitioner
Nurses

What support will you have?

We care for the whole family. You will have access to:

Child life services - To help your child feel safe and calm
Family counselors
Social workers
Spiritual care

Meet Our Team

Our doctors and nurses are experts in treating PVS. They work together to give your child the best care possible.

Follow-Up Care

PVS can come back. We keep a close eye on your child during and after treatment. You child will have:

regular check-ups.
imaging tests to watch the veins.
help with breathing, feeding, and growing.

We will be your partner for the long run.

Why Choose Children’s Mercy

Top-ranked care: Recognized for heart and lung care.
Specialized team: 1 of the few centers with a PVS program.
Family-centered: We include you in every decision.