Pulmonary valve stenosis in children

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"The vast majority of patients with significant pulmonary valve stenosis are having valve dilation in the catheterization lab, precluding the need for surgery. We are fortunate to have excellent catheterizers with high success and safety ratings, as well."

--Susan Saleeb, MD, Assistant in Cardiology, Boston Children's Hospital

Audrey Marshall, MD, is the director of Invasive Cardiology at Boston Children's Hospital.

If your infant or child has been diagnosed with pulmonary valve stenosis, a basic understanding of the condition will help you to cope with this congenital (present at birth) heart defect.

Pulmonary stenosis is a narrowing of the pulmonary valve, which normally lets blood flow from the right ventricle into the lungs.

The condition is congenital (present at birth)—and is the second most common congenital heart defect (5 to 10 percent of all cases).

  • Pulmonary stenosis often occurs by itself (in isolation), but is also a component of half of all complex congenital heart defects.
  • Pulmonary stenosis is diagnosed by a loud, harsh whooshing sound (heart murmur).
  • Mild pulmonary stenosis typically does not cause any symptoms, and generally does not require treatment.

Severe stenosis in newborns causes signs and symptoms of:

  • cyanosis (bluish tinge to skin color)
  • lethargy
  • pale, cool or clammy skin
  • circulatory shock

Symptoms in childhood are rare and may include:

  • exercise intolerance
  • heavy and rapid breathing
  • fatigue
  • Most pulmonary valve treatment is done by catheterization, rather than surgery.

Boston Children's Hospital approach to pulmonary valve stenosis

The experienced heart specialists in Boston Children’s Catheterization Lab, Congenital Heart Valve Program and Cardiac Surgery Department understand how distressing the diagnosis of pulmonary stenosis can be for parents. You can have peace of mind knowing that our specialists treat some of the most complex pediatric heart conditions in the world, with overall success rates approaching 98 percent—among the highest in the nation.

We use the following elements to provide the best possible outcomes for PVS:

  • accurate diagnosis and assessment: We utilize the most advanced techniques available for precisely determining your child’s heart anatomy, with interpretation by highly experienced cardiologists.   
  • sophisticated therapies: Boston Children’s Catheterization Lab and Congenital Heart Valve Program care for children with congenital heart defects that involve absent or malfunctioning heart valves, such as pulmonary valve stenosis. Our specialists have a strong record of excellence in heart valve repair and replacement, including minimally invasive techniques.
  • Babies with congenital heart defects often need emergency therapy. Our Cardiac Intensive Care Unit(CICU), cardiac cath labs and operating rooms deal with the urgent needs of our smallest patients 24/7.Boston  Children’s CICU was one of the first such units developed anywhere; professionals from many countries visit our CICU to learn advanced techniques of heart failure management, stabilization and post-operative care.
  • close, expert medical follow-up: If you live in the Boston area, a Boston Children’s cardiologist will follow your child after catheterization or surgical repair; if you live in another part of the US or the world, a Boston Children’s cardiologist will work closely with your local cardiologist. Adult patients with congenital heart defects are followed by Boston Children’s cardiologists with special training for adults with congenital heart problems.

At Boston Children’s, we provide families of children with PVS with a wealth of information, resources, programs and support—before, during and after your child’s treatment. With our compassionate, family-centered approach, you and your child are in the best possible hands.

Visit Heart and Blood Vessels for information on how the normal heart works.

For visual and audio information on some congenital heart defects, visit Boston Children’s Cardiovascular Multimedia Library.

Boston Children’s Cardiac Neurodevelopment Program

Children who’ve had surgery or other life-saving interventions for heart problems as infants are at greater risk of neurodevelopmental delays and difficulties. By school age, they tend to have more academic, behavioral and coordination issues than other children. Boston Children’s Cardiac Neurodevelopment Program—one of the few of its kind in the United States—provides expert screening, evaluation and care for infants, children and teenagers with congenital heart disease who are at risk for neurodevelopmental problems. Care begins soon after your child’s first cardiac procedure, and continues as your child grows to make sure she’s hitting her developmental milestones.

Pulmonary (valve) stenosis (PVS, PS): Reviewed by Susan F. Saleeb, MD
© Boston Children's Hospital; posted in 2012

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

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