Pulmonary Valve Stenosis

What is pulmonary valve stenosis?

Pulmonary valve stenosis is the term describing a narrowing in the opening of the pulmonary valve. The pulmonary valve functions as a one-way valve that allows blood to move from the right ventricle (pumping chamber) into the artery to the lungs and prevents blood from leaking back into the right ventricle.

Pulmonary valve stenosis is almost always congenital, meaning that the child is born with the narrowing of the valve. It is the second most common congenital heart defect in babies.

The pulmonary valve has three leaflets (flaps) which open as the right ventricle contracts (squeezes) to allow blood to go to the lungs and then closes as the ventricle relaxes to prevent blood from running backward (regurgitation). If this valve does not open normally, the right ventricle must work harder to develop enough pressure to push blood through the narrow opening in the valve.

Over time, the extra work for the right ventricle can result in an increased risk of heart-rhythm problems and in limitations in its ability to pump blood.

How we care for pulmonary valve stenosis

The Benderson Family Heart Center at Boston Children's Hospital is the largest pediatric heart program in the U.S. Our staff of more than 80 pediatric cardiac specialists care for thousands of children and adults with congenital and acquired heart defects each year, from simple to complex cases. We have experience treating rare heart problems with success rates that are among the best in the world.

Part of our approach to valve repair is finding new ways to get more accurate imaging information with tools such as three-dimensional (3D) modeling, echocardiograms and cardiac magnetic resonance imaging (MRI). Through use of advanced imaging, our specialists can better understand valve dysfunction and find the appropriate treatment.

Our areas of innovation for pulmonary valve stenosis

The pediatric cardiologists and cardiac surgeons at Boston Children's have pioneered several of the interventional catheterization-repair techniques, now used widely for many congenital heart defects, including pulmonary valve stenosis.

Clinical heart researchers at Boston Children’s have created a Congenital Heart Valve Program with a focus on valve repair, rather than replacement. The program has formed in response to the greater emphasis currently being placed on identifying and treating valve abnormalities in children and young adults with congenital heart disease.