Pulmonary vein stenosis (1269)

Key points below

What is pulmonary vein stenosis?

Pulmonary vein stenosis (PVS) is when veins are narrow or blocked.

heart vein

What causes pulmonary vein stenosis?

PVS happens when there is a buildup of cells inside the walls of these pulmonary veins. This causes in a narrowing or blockage. The amount of blockage and number of veins affected can be different for every child. Sometimes only a small section of the vein is narrowed or blocked. In other cases, blockages may run the length of the vein. There is not a known genetic cause.

It is not known why the buildup of cells in the pulmonary veins happens. It is also unknown why it gets worse more quickly for some children.

There are two types of pulmonary vein stenosis:

What are the symptoms of pulmonary vein stenosis?

PVS often goes overlooked and underdiagnosed. This is because the signs and symptoms can be seen in other conditions. Some are:

What tests might need to be done?

Tests to check blood flow in the pulmonary veins are:

Ask your child’s provider or nurse to learn more about these tests.

How is pulmonary vein stenosis treated?

There are three ways to treat PVS. Even after a treatment, there is a chance that PVS may return. It is important to follow up with your child’s doctor regularly.

1. Medicine treatments: There are no proven medical treatments for PVS. Different medicines may be used to manage PVS symptoms. This can include use of diuretics to help lessen fluid in the lungs. Your child may need medicines that are used
for pulmonary hypertension. After other treatments, medicines may be given to help lessen the narrowing that can re-occur.

2. Cardiac Catheterization Children may have a cardiac catheterization (or "heart cath") procedure. During the procedure, a heart doctor will put in a small balloon and inflate it. This is sometimes called a balloon angioplasty. In some cases, a small wire tube (stent) is put in to widen the vein. Both procedures help to increase blood flow through the veins.
Catheterizations are less invasive than surgery. It is common for the veins to narrow again. This means that a child with PVS may have to do this many times.

3. Surgery: Narrowed or blocked veins can be treated by a surgeon in the operating room. Surgical treatment can be done to open areas of narrowing of the pulmonary veins that are close to the heart. Opening this area helps improve blood flow through the pulmonary veins back to the heart.

Other resources that may be helpful:
#1272 Echocardiogram teaching sheet
#1433 CT Scan teaching sheet
Children’s Wisconsin webpage on Pulmonary Vein Stenosis


Call your child’s doctor, nurse, or clinic if you have any questions or concerns or if your child has special health care needs that were not covered by this information.