Pulmonary Vein Stenosis
Key Points
- Pulmonary vein stenosis is a rare heart condition in which one or more of the veins that carry blood from the lungs to the heart are narrowed.
- It may occur after surgery for certain congenital heart defects or on its own, most often in infants.
- While there is no cure, treatments like medication, cardiac catheterization and, sometimes, surgery can help to manage the condition and improve quality of life.
What are pulmonary veins, and what do they do?
The body has four pulmonary veins, which are blood vessels that carry blood from the lungs to the heart.
What is pulmonary vein stenosis?
Pulmonary vein stenosis is a rare, chronic heart condition in which the pulmonary veins are narrowed. When any of the pulmonary veins are narrowed, it becomes more difficult for the heart to receive enough oxygen-rich blood from the lungs. There are two types of pulmonary vein stenosis, primary and secondary.
Primary Pulmonary Vein Stenosis
When pulmonary vein stenosis occurs on its own, without prior surgery or intervention on the pulmonary veins, it is called primary vein stenosis. Primary pulmonary vein stenosis generally occurs in infants and young children.
Secondary Pulmonary Vein Stenosis
Secondary pulmonary vein stenosis typically occurs as a result of a separate disease or event. Most commonly, it can occur after a procedure was performed on or near the pulmonary veins, such as after a surgical repair for total anomalous pulmonary venous return. Other conditions that can cause scarring or compression within the chest, such as radiation, tumors or inflammatory diseases, can also lead to secondary pulmonary vein stenosis. Secondary pulmonary vein stenosis can occur in children or adults.
What are the causes and risk factors for pulmonary vein stenosis?
The cause of primary pulmonary vein stenosis is largely unknown, though certain risk factors can increase the potential for having the condition. These include the presence of bronchopulmonary dysplasia, which occurs when a baby’s lungs are underdeveloped due to being born prematurely, and congenital heart disease. Pulmonary disease and prematurity may also be risk factors.
What are the symptoms of pulmonary vein stenosis?
Symptoms of pulmonary vein stenosis can include:
- Rapid or shallow breathing
- Rapid heartbeat
- Fatigue
- Difficulty feeding or poor appetite
- Poor growth
- Pale skin
- Cyanosis, a blue discoloration of the skin and lips due to low oxygen
How is pulmonary vein stenosis diagnosed?
Diagnosing pulmonary vein stenosis is done using a combination of tests, typically including:
- Echocardiogram: An ultrasound done after birth that demonstrates the anatomy and flow in the heart and blood vessels.
- CT scan: Imaging of the anatomy of the heart, lungs and blood vessels in the chest.
- Cardiac catheterization: A small, flexible hollow tube (called a catheter) is inserted into a blood vessel in the groin, arm or neck to do several tests that can provide information about the structure and function of the heart. Through the same catheters, interventions can be done to the heart and surrounding blood vessels, including procedures such as balloon angioplasty (opening a blood vessel with a balloon) or stenting (placing a cylinder frame in a blood vessel to keep it open).
How is pulmonary vein stenosis treated?
While there is no permanent treatment for pulmonary vein stenosis, medication and interventions like cardiac catheterization or surgery can manage it and prevent progression of the disease. The treatment plan will depend on the patient’s individual condition, and will consider factors such as:
- whether they have other heart or lung problems
- the number of pulmonary veins affected
- the degree of narrowing in the veins
Medication
Medications may be prescribed to help prevent abnormal growth and narrowing inside the vein.
Cardiac Catheterization
Treatment often involves cardiac catheterization, in which a balloon and/or stents are used to open the narrowed veins. The stent may be coated with medication to help prevent regrowth after opening the vein.
The pulmonary veins are prone to regrowth of the lining of the vessel and the vessel wall, so frequent cardiac catheterizations are often needed to keep them open.
Surgery
Surgery in the heart to open the narrowed veins may be an option to treat pulmonary stenosis. Recurrent surgeries to reopen the narrowed veins might be needed due to the frequent narrowing of the veins.
For some patients, the only treatment option is lung transplant, which is considered in severe cases or with progression of the disease despite already having treatment.
Outlook for pulmonary vein stenosis
Outcomes for pulmonary vein stenosis have improved greatly over the years. With treatment, many patients can have improved quality of life and longer lifespans.
Without treatment, pulmonary vein stenosis will worsen and can lead to pulmonary hypertension (high blood pressure in the lungs) and heart failure. Pulmonary vein stenosis often will recur, and some children will need close monitoring from their care team and repeated interventions, such as cardiac catheterization or surgery, throughout their lives.
Medically reviewed by Preston Boyer, M.D., April 21, 2026
