Peripheral pulmonary stenosis

Hearing the term peripheral pulmonary stenosis applied to your child can frightening and hard to accept. You may find yourself struggling with a seemingly endless list of questions: What caused this condition? How will it change my child's life? Is surgery necessary? How will this diagnosis affect my family? Where do we go from here? 

Learning more about peripheral pulmonary stenosis—which is also sometimes referred to as "pulmonary branch stenosis" or "branch pulmonary artery stenosis"—can help you better understand what's next and what to expect. 

The following pages will introduce you to the basics about the condition.

• Peripheral pulmonary stenosis refers to narrowing in one or several areas of the branches of the pulmonary arteries. This is a different condition from pulmonary (valve) stenosis (PVS, PS) and pulmonary vein stenosis.
• Peripheral pulmonary stenosis may be a progressive condition, meaning symptoms can increase and become more obvious and disruptive over time.
• Many children in the mild stages of peripheral pulmonary stenosis do not show any outward symptoms.
• Sometimes, peripheral pulmonary stenosis occurs as a complication of rare heart defects or certain genetic syndromes.
• It can also occur if a child is born without a pulmonary valve.
• Children with more severe peripheral pulmonary stenosis are likely to need interventional catheterization. Most do very well after undergoing this type of procedure.
• Children with peripheral pulmonary stenosis are at risk of having insufficient oxygen within their blood, placing increased pressure on the heart. This makes the heart have to work harder.
• Peripheral pulmonary stenosis can affect each child differently. Your physician is the best resource for providing detailed information about your child’s individual situation. Your clinician and treatment team will recommend the treatment options that best meet the needs of your child and your family.

How Children’s Hospital Boston approaches peripheral pulmonary stenosis

The Children’s Hospital Boston Cardiovascular Program team has extensive experience treating children, adolescents and adults with peripheral pulmonary stenosis.

Our specialized training in pediatric cardiology means that we understand the particular challenges, circumstances and intricacies of working with young people with heart problems. In addition to our medical expertise, we provide patient-centered care that always recognizes your child as an individual—and we offer resources to meet the needs of your entire family.

• With more than 80 cardiac experts on our staff, Children’s operates the largest pediatric heart program in the nation.
• We use the most sophisticated diagnostic and imaging procedures, including interventional catheterization,and offer dozens of specialized services in such areas as heart valve replacement, cardiac anesthesia, robotic surgery and fetal cardiology.
• Our Department of Cardiology and Department of Cardiac Surgery clinicians work closely with you to determine the right treatment plan for your child. We consider you an invaluable member of the treatment team, and always welcome your input and questions.

Peripheral pulmonary stenosis: Reviewed by Doff McElhinney, MD
© Children’s Hospital Boston, 2010