What is Pulmonary Vein Stenosis (PVS)?

What is Pulmonary Vein Stenosis (PVS)?

Pulmonary vein stenosis (PVS) is a rare and progressive disease affecting newborns and children of all ages. Even with aggressive treatment, PVS is a potentially lethal disease with no known cause or cure.

In pulmonary vein stenosis, the pulmonary veins – which move oxygen-rich blood from the lungs to the heart –become increasingly narrowed. The veins continue to narrow until they are completely closed off. As a result, the right side of the heart struggles to pump blood into the lungs and the lungs become congested, which can lead to heart failure.

Diagram of a healthy heart and a heart with pulmonary valve stenosis depicting the narrowing of a pulmonary valve.

Some children may only have pulmonary vein stenosis in one pulmonary vein, while others may have multiple veins affected, in either one or both lungs. Regardless of the number of veins involved, PVS usually will worsen in the affected veins over time and spread to other veins that were previously normal, making the condition difficult to manage and treat.

Types of Pulmonary Vein Stenosis


There are two types of pulmonary vein stenosis:

  • Primary pulmonary vein stenosis (PVS), or congenital pulmonary vein stenosis, occurs in children with no prior surgery on the heart or the pulmonary veins. Primary PVS often develops in infants born prematurely or with lung inflammation from reflux, although the latter is not a common cause.
  • Secondary pulmonary vein stenosis (PVS) occurs in infants and children after surgical repair of the pulmonary veins due to congenital heart disease (CHD).

Signs & Symptoms of Pulmonary Vein Stenosis


The symptoms of pulmonary vein stenosis can vary in children from no signs to a variety. These include:

  • Fast breathing (tachypnea)
  • Fatigue or lack of energy
  • Poor weight gain
  • Pale or bluish skin tone
  • Frequent colds or chest infections
  • Bleeding in the lungs (coughing up blood)

What Causes Pulmonary Vein Stenosis?


The exact cause of pulmonary vein stenosis is unknown. It’s usually the result of a congenital heart defect, which occurs when a baby is developing in the womb.

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Trust NewYork-Presbyterian for Pulmonary Vein Stenosis Care

Children with pulmonary vein stenosis need specialized care like that found at NewYork-Presbyterian, where our multidisciplinary team of pediatric specialists focuses on treating children with this complex condition.

We care for you promptly, thoroughly, and with the thoughtfulness and compassion you and your child deserve. As a parent or caregiver, we consider you part of your child’s team. We encourage you to ask questions and let us know how we can support you and your family.