Herma Heart Institute

Pulmonary vein stenosis

Pulmonary vein stenosis (PVS) is a rare disease that requires a high degree of management and care. Different from pulmonary valve stenosis, pulmonary vein stenosis is the narrowing or blocking of the pulmonary veins. Because PVS is so unique and rare, it requires a level of expertise and coordination found at only a handful of centers. Learn what to look for in a center.

What is pulmonary vein stenosis?

In a structurally normal heart, there are four pulmonary veins that carry oxygen-rich blood from the lungs to the heart. This blood is then pumped out to the rest of the body to provide the oxygen it needs to function.

Pulmonary vein stenosis occurs when there is a buildup of cells inside the walls of these pulmonary veins, resulting in a narrowing or blockage. The severity of the blockage can vary from child to child. Sometimes, only a small section of the vein is narrowed or blocked, whereas more severe cases have blockages that run the length of the vein.

There are two types of pulmonary vein stenosis: primary pulmonary vein stenosis and post-repair stenosis.

Primary PVS: Also known as congenital pulmonary vein stenosis or idiopathic pulmonary vein stenosis, this type of PVS occurs when there has not been a surgery or procedure on the pulmonary veins. This type of PVS is often observed in babies who were born very prematurely.
Post-repair PVS: This type occurs following a procedure or surgery where the pulmonary veins have been repaired, such as when treating total anomalous pulmonary venous return (TAPVR). Currently, published research shows that post-repair PVS is observed in roughly 10% of the post-operative TAPVR patients.

Why is pulmonary vein stenosis a concern?

Pulmonary vein stenosis is often a progressive disease. This means that over time, the disease can spread to other veins that were previously healthy. A severe narrowing or blockage of the pulmonary veins can ultimately result in pulmonary hypertension and/or heart failure.

What are causes and risk factors of pulmonary vein stenosis?

There is a great deal unknown with pulmonary vein stenosis. It is not known why the buildup of cells in the pulmonary veins occurs, nor is it known why the condition progresses more rapidly for some children more than others. To date, there is no genetic cause identified for pulmonary vein stenosis.

For children who undergo a procedure related to repairing the pulmonary veins, such as total anomalous pulmonary venous return (TAPVR), there is an increased likelihood of post-repair PVS.

What are signs and symptoms of pulmonary vein stenosis?

Pulmonary vein stenosis often goes overlooked and underdiagnosed as the signs and symptoms are broad. Below are a few symptoms that can be associated with pulmonary vein stenosis:

Rapid breathing
Fatigue
Failure to thrive, difficulty gaining weight or delayed growth
Frequent colds and pneumonia
Bleeding in the lungs

How is pulmonary vein stenosis diagnosed?

Pulmonary vein stenosis often goes undetected, and is only found when a child has a test for another reason. Some tests that are used to observe blood flow in the pulmonary veins are:

How is pulmonary vein stenosis treated?

There are three approaches for pulmonary vein stenosis treatment. Even after a treatment is performed, there is the possibility that PVS may return and therefore routine follow-ups are essential.

Medical treatments: A variety of medications may be used to manage pulmonary vein stenosis. This can include medications that are commonly used for pulmonary hypertension, or diuretics to help minimize fluid in the lungs.
Catheterizations: Children may undergo a catheterization (or "cath") procedure to address the narrowed veins. During the procedure, an interventional cardiologist will insert and inflate a small balloon to temporarily expand the vein. While the vein is propped open, a miniscule metal structure (stent) is placed to widen the vein and help to increase blood flow. While catheterizations offer a less-invasive solution to narrowed or blocked veins, it is common for the veins to eventually narrow again. This means that a child with PVS will often have many catheterizations to address the narrowing of their pulmonary veins.
Surgery: Extremely narrowed or blocked veins can also be treated by a surgeon in the operating room. Surgical treatment is also often used as the first intervention for pulmonary vein stenosis. The surgeon will open the pulmonary veins to remove the cell build up and allow for blood flow.

What to look for in a center to manage PVS

Pulmonary vein stenosis is a rare disease that requires ongoing management by a team of experts from multiple disciplines. Because PVS is often a progressive disease that requires multiple cath and/or surgical interventions, it is essential that team members from pediatric cardiology, interventional cardiology and cardiothoracic surgery collaborate together on an individual patient's options. Given the rarity of pulmonary vein stenosis, the team should be engaged in the latest research and studies related to PVS, and collaborate with other PVS centers on how to best manage care.

The Herma Heart Institute has a dedicated Pulmonary Vein Stenosis group that monitors and discusses care for all patients diagnosed with pulmonary vein stenosis. This group comprises of pediatric cardiologists specialized in pulmonary hypertension, interventional cardiologists (cardiac catheterization), and cardiothoracic surgeons who stay on top of the latest research related to pulmonary vein stenosis management. Together, this team meets at least monthly to review and discuss patient cases and provide a coordinated, thoughtful approach for each child living with pulmonary vein stenosis. The team can also meet more frequently based on new patients and patient needs.

The Herma Heart Institute is part of the Pulmonary Vein Stenosis Network, an international multi-center registry that compiles clinical data on patients living with pulmonary vein stenosis. This collaboration on pulmonary vein stenosis allows for centers to share best practices and produce better outcomes.