Congenital Pulmonary Valve Stenosis

Congenital pulmonary valve stenosis is a condition in which a baby is born with a pulmonary valve that is too small, narrow or stiff.

Symptoms of pulmonary stenosis depend on the size of the narrowed pulmonary valve. If symptoms are mild, pulmonary stenosis may not require treatment. Children with more severe pulmonary stenosis will need a procedure to fix the pulmonary valve so blood can flow properly to the lungs. If the stenosis is extreme, a baby may need an emergent procedure to open the valve.

The pulmonary valve controls the flow of blood as it leaves the right ventricle of the heart and keeps it flowing forward to the lungs. It opens to let blood move ahead, then quickly closes to keep it from flowing backward.

In congenital pulmonary valve stenosis, the pulmonary valve is too small or too narrow and can’t open all the way. This causes the right ventricle to pump harder to send blood out to the lungs. Over time, this can cause the right ventricle to thicken and strain the heart.

The specialists at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine — the leading providers of pediatric heart care in Louisville and Southern Indiana — can help your child with congenital pulmonary valve stenosis.

The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the skills and experience to provide a pinpoint diagnosis and know when pulmonary stenosis requires surgery and when other treatment options can be pursued.

Norton Children’s has a network of outreach diagnostic and treatment services throughout Kentucky and Southern Indiana.

A diagram shows congenital pulmonary valve stenosis in comparison to a healthy child's heart

Pulmonary Stenosis Symptoms

Many people with pulmonary stenosis have no symptoms. Others have mild symptoms that usually don’t become bothersome.

Symptoms of severe pulmonary stenosis can include:

  • Being very tired
  • In newborns, a bluish tint to the skin (cyanosis) caused by blood that is low in oxygen
  • Chest pain
  • Fainting
  • Palpitations (sensation of rapid or irregular heartbeat)
  • Poor weight gain
  • Shortness of breath
  • Swollen abdomen

In babies, pulmonary stenosis happens when a baby’s heart doesn’t develop the way it should during pregnancy. Doctors don’t know why this happens. The cause of congenital pulmonary valve stenosis is unknown.

Diagnosing Congenital Pulmonary Valve Stenosis

Doctors can identify pulmonary stenosis before birth. This allows treatment to occur immediately after birth. A fetal echocardiogram (fetal echo) uses sound waves to create a moving picture of the heart. This helps doctors see how the baby’s heart looks and works before the baby is born.

Norton Children’s Heart Institute offers fetal echo tests at six sites in the cities of Ashland, Lexington, Louisville, Owensboro and Paducah to examine the structure of your baby’s heart.

In other cases of pulmonary stenosis, the doctor may hear a heart murmur — a specific “whooshing” sound — because of the uneven blood flow through the narrowed pulmonary valve. Your child then may be sent to a pediatric cardiologist. To diagnose your child’s condition, the pediatric cardiologist may use:

  • Echocardiogram (echo): This test uses sound waves (ultrasound) to produce images of the heart and blood vessels’ structures on a screen. It can show structure of the heart and ductus arteriosus and also the function of the heart. Norton Children’s Heart Institute has 28 tele-echo locations throughout Kentucky and Southern Indiana.
  • Chest X-ray: This shows pictures of the heart and lungs, and can show heart and lung issues, including extra blood flow or fluid in the lungs.
  • Electrocardiogram (ECG or EKG): A test that checks the heart’s electrical activity to show damage or irregular rhythms.
  • Heart catheterization: This invasive procedure studies the structure, function, and provides direct pressure measurements of the heart.

Congenital Pulmonary Valve Stenosis Treatment

In some cases, pulmonary stenosis doesn’t need to be treated. In severe cases, the pulmonary valve will need to be fixed or replaced.

Many types of procedures can be used to repair or replace the severely stenosed pulmonary valve. Some cases of pulmonary stenosis can be treated with a balloon valvuloplasty during heart catheterization. With this procedure, a doctor threads an unopened balloon through the pulmonary valve and inflates it to open the valve.

Other children may need a valve replacement, which involves implanting an artificial valve or a valve from a donor.

To decide what treatment to use, doctors consider:

  • Child’s age and size
  • How well other valves in the heart are working
  • Location of the narrowing and the amount of narrowing
  • Whether the child has had previous heart surgery
  • Whether the child has other medical conditions

Your pediatric cardiologist and pediatric cardiothoracic surgeon at Norton Children’s Heart Institute will discuss valve replacement options in order to find which option is best for your child.

Pulmonary Stenosis Complications and Follow-up

Following treatment for pulmonary stenosis, the child will continue to follow-up with their pediatric cardiologist. Reasons for this include reoccurrence of pulmonary stenosis from scar tissue that forms after a valvuloplasty or a valve replacement that doesn’t grow as the child gets bigger. Some children might need several procedures in their lifetime to keep the valve functioning properly.

After treatment, most children can enjoy regular activities after their recovery. Children and teens with moderate or severe pulmonary stenosis should talk with their cardiologist before playing competitive sports or being very physically active.

Why Choose Norton Children’s Heart Institute

The Adult Congenital Heart Association has recognized Norton Children's Heart Institute for its expertise treating adult congenital heart disease.

  • Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
  • The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
  • The Adult Congenital Heart Association has accredited Norton Children’s Heart Institute’s Adult Congenital Heart Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
  • More than 5,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
  • Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
  • The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.
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