Pulmonary Valve Stenosis (Tetralogy of Fallot)

Pulmonary valve stenosis (also known simply as pulmonary stenosis) is a condition that affects the pulmonary valve, which connects the heart’s lower right chamber to the lungs.

Blood flows through the pulmonary valve on its way to the lungs, where it receives oxygen before it’s pumped to the rest of the body. But in pulmonary stenosis, the pulmonary valve may be too small, narrow or thick, and the flaps that make it open and close may not be formed properly. As a result, it can’t open all the way, and blood can’t flow through the way it’s supposed to. This strains the heart, which has to work harder than it should to pump blood to the lungs.

This condition occurs as part of a group of heart defects called tetralogy of Fallot, including a ventricular septal defect and, often, an abnormal pulmonary artery.
 

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What Are the Signs and Symptoms of Pulmonary Valve Stenosis?

Many children and adults with pulmonary stenosis only have mild signs or symptoms, or none at all. More serious symptoms include:

  • Bluish or greyish lips, skin and nails
  • Heart murmur
  • Heart beat that’s too fast, too slow or irregular
  • Trouble breathing
  • Chest pain
  • Feeling dizzy or lightheaded
  • Feeling very tired
  • Passing out
  • Swelling in belly
     

What Causes Pulmonary Valve Stenosis?

Pulmonary stenosis usually occurs before birth, when the baby’s heart doesn’t develop properly during pregnancy.

In some babies, it may be linked to one of these risks.

  • Genetic condition like DiGeorge syndrome or Noonan syndrome
  • Family history of congenital heart problems
  • Mother had an infection like rubella (German measles) during pregnancy
  • Mother used certain harmful medications during pregnancy

But pulmonary stenosis also occurs in babies without any of the above risk factors. For these patients, there’s often no known cause.

How is Pulmonary Valve Stenosis Treated?

If a case of pulmonary stenosis is mild, it may not need any treatment. But it’s important to check in regularly with a heart doctor (cardiologist) to make sure it’s not getting worse.

In more serious cases, doctors may fix or replace the pulmonary valve. Connecticut Children’s Heart Center specializes in a range of techniques, from minimally invasive cardiac catheterization to congenital heart surgery. Our experts work together to determine which approach is best for each patient.

  • Treat symptoms with medicine
  • Balloon valvuloplasty: Uses a long, thin tube (catheter) to place a balloon in the pulmonary valve, and inflate it to open the valve
  • Transcatheter pulmonary valve replacement: Replaces the pulmonary valve using a catheter
  • Pulmonary valve repair: Open-heart surgery to reconstruct or reshape the pulmonary valve
  • Pulmonary valve replacement surgery: Open-heart surgery to replace the pulmonary valve

Patients born with pulmonary stenosis often need to follow up with congenital heart experts throughout their life, including with regular tests like echocardiograms or cardiac magnetic resonance images (MRIs). If their valve was replaced when they were young, they may need additional surgeries or catheter procedures as they grow.

Connecticut Children’s Heart Center is there every step of the way. Our experts understand the unique anatomy of the heart after congenital surgeries or procedures. We’re proud to continue caring for patients in adulthood with our nationally accredited Adult Congenital Heart Disease program.

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