Understanding Pulmonary Vein Stenosis
The heart pumps blood to the lungs to pick up oxygen. This oxygen-rich blood then travels back to the heart through the pulmonary veins. Pulmonary vein stenosis (PVS) occurs when one or more of these veins becomes narrowed or blocked, making it harder for blood to return to the heart. This, in turn, increases pressure in the lungs leading to long-term complications. PVS is a rare, serious disease that sometimes goes unrecognized. It often affects babies born prematurely or with other cardiac conditions. However, it can also affect healthy individuals.
Comprehensive and Personalized Treatment of PVS
Due to the complexity of PVS and the need for specialized care, the Pulmonary Vein Stenosis Program was created as part of the Heart Institute at Nicklaus Children's Hospital. This is the only program in Florida offering a team of experts focused on treating PVS.
Since every case of PVS is unique, our team carefully evaluates each patient's condition and symptoms to create a personalized treatment plan. Treatment plans can include medications, minimally invasive procedures like cardiac catheterization, or surgery.
Multidisciplinary Team of Specialists
The Pulmonary Vein Stenosis Program is comprised of multidisciplinary team of specialists, who work collaboratively to offer individualized care for each patient. The team includes:
- Cardiologists
- Cardiac Imaging specialists
- Pulmonary hypertension specialists
- Neonatologists,
- Hematologist-oncologists
- Interventional cardiologists
- Cardiothoracic surgeons
Conditions we Treat
The Pulmonary Vein Stenosis Program offers care for children with all forms of PVS, including:
- Intraluminal pulmonary vein stenosis: This form of PVS is linked to an overgrowth in connective tissue cells within the lumen, which is the central opening of a blood vessel. The overgrowth causes abnormal narrowing of the pulmonary veins.
- Isolated pulmonary vein stenosis: This form of pulmonary stenosis occurs without any other heart or lung conditions. Often affected babies appear healthy for several weeks before developing breathing difficulties and experiencing low oxygen levels.
- Pulmonary vein stenosis as a secondary complication: In children, PVS can occur as a complication associated with another heart or lung condition, such as congenital heart disease or chronic lung disease. Often the PVS is discovered following the earlier heart or lung disorder diagnosis.
Treatments and Procedures
Nicklaus Children’s offers a wide range of diagnostic methods and treatments for patients with pulmonary vein stenosis. These include:
Diagnosis and Assessment Procedures:
- PVS is typically diagnosed with one or more of the following procedures or technologies.
- Echocardiography: This method uses sound waves to create moving images of the heart’s structure and function.
- Cardiac computed tomography (CT): This noninvasive procedure uses X-ray equipment and computers to produce images of the pulmonary veins.
- Pulmonary blood flow scan: This approach uses an injectable dye and special camera to track the flow of blood through a child’s lungs.
Treatment Methods:
- Balloon Angioplasty and Stent Angioplasty: These two methods are among care options for children with pulmonary vein stenosis. While the child is sedated, a thin plastic tube is inserted in the groin and advanced to the affected vein or artery. A balloon attached to the end of the catheter is inflated at the site of the blockage to restore blood flow. In some cases, a stent (a small mesh-like tube) is placed across the vein to hold it open. This is known as stent angioplasty.
- Open-heart surgery: Some children with PVS may require open-heart surgery to widen areas of the pulmonary veins that have narrowed, restoring normal blood flow.
- Medication therapy: Among the tools available to the care team are medications that can slow or stop the recurrence of pulmonary vein stenosis.
How to Get Started
To make an appointment with the Pulmonary Vein Stenosis Program, please contact the Heart Institute nurse navigator.
As a patient navigator for the Heart Institute, Jessica Quinones serves as the primary point of contact for families and referring physicians whose infants have been diagnosed with a heart defect. She aids in securing appointments with specialists, scheduling and explaining diagnostic tests, and procedures.