Pulmonary vein stenosis

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Pulmonary vein stenosis (PVS) is when there is a narrowing or blockage in the pulmonary veins. It is a rare disease and if it progresses, it can lead to pulmonary hypertension and right heart failure.

Key points

Each child is unique. The number of pulmonary veins affected and where the stenosis is located is not the same in every child.
There are two main categories: primary PVS and post-repair PVS. Primary PVS is when the child develops PVS without having any surgery on the pulmonary veins, while post-repair PVS develops after surgical repair of the pulmonary veins.
Progression of the disease can be clinically silent, meaning there are no symptoms.
PVS is a rare but very serious disease.
Children can have surgery or catheter-based interventions (balloon angioplasty or stents) to help relieve the narrowing and help blood flow better through the lungs.

Introduction

Pulmonary vein stenosis (PVS) affects the pulmonary veins. The pulmonary veins bring blood from the lungs back to the left atrium of the heart. In PVS, the pulmonary veins become narrowed. In some cases, the narrowing can cause the pulmonary veins to become blocked. This is caused by an abnormal build-up of cells inside the wall of the pulmonary vein. It is not known what causes the cells to build-up. PVS is a rare but very serious disease.

If multiple pulmonary veins are affected, it can cause pulmonary hypertension. As a result, the right heart has to work harder to pump blood through the lungs. If the lung pressure remains high, it may result in right heart failure.

View of the inside of the heart with blood entering left atrium from the pulmonary veins of the both lungs — Typically, there are four pulmonary veins, two from each lung. These veins drain oxygen-rich blood from the lungs into the left atrium of the heart. This blood is then moved out into the body, supplying it with the oxygen needed for proper health and function.

More information

What is PVS

Narrowing or blockage can affect any of the pulmonary veins. The narrowing is not the same in all children. In some children, only a small part of the vein will be narrowed or blocked. Others may have long segments of the vein blocked or even the entire vein.

Some children may only have PVS in one pulmonary vein. Other children may have multiple veins affected, in either one or both lungs.

Illustration of small part of vein narrowed and large part of veins narrowed — Pulmonary vein stenosis is a narrowing or, at times, a blockage of one or more of the four pulmonary veins. Narrowing or blockage can occur in a small part of one of the veins or in larger parts of one or more veins. This reduces the amount of blood that is able to drain from the lungs back into the heart.

There are two main categories of PVS:

Primary, or congenital, PVS can occur in children who do not have a history of heart disease.
Post-repair PVS can develop after a child has surgery to repair anomalous (abnormally connected) pulmonary veins.

Signs and symptoms

Signs and symptoms of PVS

PVS can be clinically silent. This means a child might not have any symptoms of PVS until the disease is very advanced.

Some signs and symptoms of PVS may include:

Fast breathing (tachypnea)
Fatigue or lack of energy
Some babies do not gain any weight, because their heart is working harder to pump blood through the lungs
Frequent colds or chest infections
Bleeding in the lungs

None of the above signs or symptoms are specific to PVS.

Causes, risk factors and prevalence

Understanding PVS

The cause of PVS is not known. It is also not known why some children develop PVS that progresses, while others have PVS that remains more stable. No genetic cause for PVS has been identified.

Children who have more serious PVS are those who:

have multiple pulmonary veins involved
have both lungs involved
have other heart defects
are diagnosed at a very young age
have recurrent disease, meaning PVS continues to come back, despite treatment.

Children who had a repair of anomalous pulmonary veins as an infant have around a 10% risk of developing pulmonary vein stenosis.

Diagnosis

Diagnosing PVS

Because PVS can be clinically silent early on, many children will have a diagnosis of PVS made when they have tests for other reasons.

Children who are suspected of having PVS will have an echocardiogram to look at the blood flow in the pulmonary veins and how well the right heart is pumping.

Your child’s doctor will also confirm the diagnosis with a CT scan, MRI or diagnostic catheterization. Some health-care centres use lung perfusion scans to help diagnose PVS. These tests let the doctors understand how many pulmonary veins are affected and how narrow the veins are. These tests are important to help plan the treatment of PVS.

Treatment

Treatment for PVS

Surgery

Surgery can be done to relieve the narrowing (stenosis) in the pulmonary veins close to the atrium of the heart. The surgeon will relieve blocked areas of the pulmonary veins. This allows blood to flow unobstructed through the veins. Your child will be given a general anaesthesia to put them to sleep during the surgery.

Catheter-based interventions

In most cases, if only one pulmonary vein is affected, it may be treated with catheter-based interventions. These include stent placement and balloon angioplasty. Both the stent and the balloon will open the area of stenosis and allow blood to flow through the veins.

One of the risks of catheter-based interventions is restenosis. This means that it is possible for narrowing of the pulmonary veins to redevelop in the future.

If the pulmonary vein is too narrowed to place a catheter through, surgery may be required.

Other treatment options

A lung transplant may be an option for some children if they have progressive disease that cannot be treated by surgery or catheter-based interventions.

There are no proven medical therapies to treat PVS, but there are experimental therapies that are currently being tested. Your child may be given specific medications to help manage the symptoms of PVS.

Complications

Complications of PVS

Children with PVS may experience some complications including:

Recurrent colds or infections that may require hospitalization
Some children may cough up blood from their lungs (hemoptysis) due to the high pressures in the lungs.

Even after treatment, it is possible that PVS may recur (come back). It is important that your child undergo imaging tests of the pulmonary veins after any treatment to monitor stenosis.

Progressive PVS can lead to pulmonary hypertension and right heart failure.

When to seek medical attention

When to see a doctor

You should see a doctor if:

your child is breathing very fast
your child is not growing and keeping up with their peers
your child develops repeated serious chest infections or coughs up blood.

At SickKids

If your child is coming for surgery at SickKids, find important information here: Coming for surgery

For more information on the Cardiology Clinic at SickKids: Cardiology Clinic

Resources

PVS Network: http://www.pvsnetwork.org

References

M. Samanek, M. Voriskova. Congenital heart disease among 815,569 children born between 1980 and 1990 and their 15-year survival: a prospective Bohemia survival study Pediatric Cardiology, 20 (1999), pp. 411–417

Last updated: March 22nd 2018