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Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), most children born with a heart problem — even newborns only hours or days old — can be quickly diagnosed and treated right when it matters the most. At the Cardiac Center, we specialize in early detection and repair of congenital heart defects (also often called, “congenital heart disease”).
If your child’s been diagnosed with pulmonary stenosis, we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.
Pulmonary stenosis refers to a narrowing (“stenosis”) of the:
- pulmonary valve
- structures immediately above the valve in the main pulmonary artery (“supravalvar”)
- structures immediately below the valve in the right ventricle (“subvalvar,” also known as “right ventricular outflow tract obstruction”)
Normally, the oxygen-poor (“blue”) blood is pumped from the right ventricle (the lower right chamber of the heart that receives oxygen-poor blue blood from the right atrium) through the pulmonary valve and out the pulmonary artery to the lungs to be oxygenated.
With pulmonary stenosis, the right ventricle must work harder to pump all of the blood through the narrowed (or “stenotic”) region. Over time, this causes thickening of the right ventricular wall (this is called “hypertrophy”), which may make narrowing of the subvalvar area worse as the thick muscle further blocks the pathway for blood.
How Does the Heart Normally Work?
When your child has a congenital heart defect, there’s usually something wrong with the structure of the heart. In order to understand your child’s condition, it can help to know how the heart should work normally.
Learn More About Normal Cardiac Anatomy »
Nemours’ experts at KidsHealth.org also offer these helpful resources to help both you and your child understand how the heart works:
Children with an extremely narrow pulmonary artery may be very ill in the newborn period (this is called “critical pulmonary stenosis”). After the newborn period, most cases of pulmonary stenosis are found when a caregiver hears a murmur, although some children may also have symptoms of trouble breathing or poor exercise tolerance (difficulty exercising). But some children may make it into adolescence or adulthood without being diagnosed. These patients may develop symptoms of right ventricular failure, with an enlarged liver and fluid retention in the legs (called “edema”) or abdomen (called “ascites”).
At the Nemours Cardiac Center, we can usually diagnose pulmonary stenosis using an echocardiogram (or “echo”). An echo is a completely safe and painless test that uses ultrasound (sound waves) to build a series of pictures of the heart. Further testing usually isn’t necessary, although many children will have a cardiac catheterization as part of their treatment.
The standard treatment for pulmonary valve stenosis is a procedure called a “balloon valvuloplasty,” which involves inserting a heart catheter through a vein in the leg leading to the heart. A balloon at the tip of the catheter is positioned within the stenotic valve and inflated to stretch open the valve.
If the balloon procedure is unsuccessful or, in cases where there may be narrowing either above or below the valve, children may need open-heart surgery.
Surgical treatment for pulmonary stenosis involves either:
- treating the valve itself
- sewing a patch onto the pulmonary artery or right ventricular outflow tract to enlarge the pathway for blood going to the lungs
Depending on the severity of the obstruction and the treatment method, about 10 to 15 percent of children will need additional interventions to enlarge the valve as they grow to adulthood. And following the procedure, one-third to one-half of children will have significant leakage of the valve. But children usually tolerate this well — and most won’t need an operation or procedure to fix the leakage. In fact, most children will be as active and able to exercise as children without heart defects.
If your child has pulmonary stenosis, know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care. Our goal is to guide your family, from start to finish, through your child’s heart defect journey — and to help your child live the healthiest, most fulfilling life possible.
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Information for Patients
Outpatient Services and Inpatient Units: (302) 651-6660
After 5 p.m. and Weekends:
Cardiac Intensive Care Unit: (302) 651-6644
General Inpatient Unit, 2B: (302) 651-6690