Pulmonary Vein Stenosis in Children

What is pulmonary vein stenosis (PVS)?

Pulmonary vein stenosis (PVS) is a rare condition where blood vessels carrying oxygen-rich blood from the heart to the lungs become narrowed or blocked. As a result, it's harder for blood to move through the lungs, which can lead to breathing problems, elevated pressure in the pulmonary artery and heart failure.

The causes of PVS aren't fully understood. Some children are born with the condition. Others, especially those born very prematurely, can develop it in their first year of life. PVS can also develop in children after heart surgery, especially if the pulmonary blood vessels were involved.

Our approach to PVS

UCSF Benioff Children's Hospitals has the only program in California and neighboring states dedicated to treating PVS. As such, we care for one of the country's largest populations of children with this complex condition. Our Pulmonary Vein Stenosis Program brings together a multidisciplinary team of internationally recognized specialists with expertise in caring for children with PVS. We provide screening, observation and treatment, including catheter-based procedures, surgery and medication.

Our team works with experts in pulmonology, pulmonary hypertension, interventional cardiology and cardiothoracic surgery. We're one of the few programs in the nation with the infrastructure for a comprehensive, multidisciplinary approach to this condition. This collaborative approach ensures that each child receives a personalized, individualized care plan.

UCSF also is actively contributing to the growing body of research on PVS. We have multiple active research studies, including PVS outcomes research, imaging studies, clinical trials of medical therapy for associated conditions such as pulmonary hypertension, and analysis of new interventional techniques. The program collaborates with the Pulmonary Vein Stenosis Network, an international research consortium that collects data from centers across the United States and Canada.

While PVS can't be cured, advances in treatment help our patients live longer and better lives.

We offer both in-person and telehealth visits and try to schedule tests and procedures for the same visit when possible.

Signs and symptoms of PVS

PVS can occur suddenly or progress gradually over time. Common symptoms include:

• Trouble breathing
• Coughing
• Fatigue
• Poor weight gain
• Blue-tinged appearance of the skin, lips or nail beds

Diagnosis of PVS

PVS can be hard to detect because symptoms are often mild at first or resemble other lung or heart problems. To determine if your child has PVS, the doctor may recommend the following tests:

• Echocardiogram: This type of ultrasound uses sound waves to create images that show blood flow in the heart and veins.
• CT or MRI scans: These are two different types of imaging tests that provide detailed pictures of the heart and blood vessels.
• Cardiac catheterization: Guided by imaging, a doctor threads a thin tube through the blood vessels into the heart to measure blood pressures and look directly at the veins. This is often the most accurate way to confirm PVS.
• Pulmonary flow scans: This imaging test shows how much blood flow is going to each area of the lungs.

Treatment of PVS

The treatment approach for PVS depends on a child's condition. The doctor will consider how many veins are affected, the severity of the narrowing and where it's located, as well as other factors. Possible treatments include:

• Observation: If only one vein is mildly narrowed and there are no symptoms, careful monitoring may be enough.
• Catheter-based procedure: This minimally invasive technique allows the doctor to use balloons or stents (tiny tubes) to open up narrowed veins.
• Surgery: In some cases, surgery is needed to repair the veins. Some children with severe disease may require a lung transplant.
• Medication: Some medicines, like sirolimus (Rapalune), may help slow down the narrowing, especially in children with severe disease.

In all cases, the goal of treatment is keeping the veins open and managing symptoms, which often requires regular follow-up. In some cases, treatments need to be repeated.