Pulmonary Vein Stenosis

Also known as: PVS

What is pulmonary vein stenosis?

Your heart pumps blood to your lungs to pick up oxygen. This oxygen-rich blood then travels back to your heart through the pulmonary veins. In pulmonary vein stenosis (PVS), one or more of these veins become narrowed or blocked, making it harder for blood to flow smoothly and increasing pressure in your lungs.

PVS is a rare and often unrecognized yet serious disease that causes a blockage of the blood vessels that bring the blood from the lungs back into the heart. It often affects babies born prematurely or with other cardiac conditions, however, it can also affect otherwise healthy patients.

What causes pulmonary vein stenosis?

The exact cause of PVS is unknown and can vary by case. Often times, PVS may occur due to a congential (occurring from when a baby is developing in the womb) heart defect. In other cases, some children can develop PVS as a secondary complication of another heart or lung issue. 

What are the symptoms of pulmonary vein stenosis?

PVS symptoms can vary from none to a variety and can change suddenly. Some symptoms of PVS may include:

  • Shortness of breath
  • Rapid heartbeat
  • Fatigue
  • Lack of appetite or poor weight gain
  • Pale or washed out skin tone
  • Rapid breathing (tachypnea)
  • Coughing up blood

What are pulmonary vein stenosis care options?

Due to the rareness and complexity of PVS, every case is unique and requires specialized care. Care options can include medications, minimally invasive procedures like cardiac catheterization, or surgery, depending on the severity of your child's condition. Nicklaus Children's Hospital is the only pediatric hospital in Florida with a Pulmonary Vein Stenosis Program. Our PVS program is part of the Heart Institute at Nicklaus Children's Hospital and brings together a wide range of specialists to provide our patients with comprehensive care.


Reviewed by: Daniel Duarte Caceres, MD

This page was last updated on: July 31, 2024 12:07 PM

The Heart Institute: Pediatric Cardiology and Cardiovascular Surgery

The Heart Institute – a world leader in pediatric cardiology and cardiovascular surgery and the care of children with congenital heart disorders – serves as a beacon to families confronting the reality of a child or newborn with a heart defect.

Learn More