Pulmonary Vein Stenosis Program

For children with pulmonary vein stenosis and their families, the severity of the disease, which is progressive and often fatal, is made worse by the search for an experienced doctor who can treat it. Since this rare disease affects very few children in the United States each year, most doctors have never seen a child with the disease.

At Children’s Hospital Boston, we’re fortunate to have a team of cardiac specialists who have been caring for children with pulmonary vein stenosis and have been studying the disease for decades.

Our expertise
Our Pulmonary Vein Stenosis team bases treatment on the latest clinical research. At Children’s, we have one of the world’s most extensive research programs and our scientists work directly with our doctors so that the newest treatments are always available.

• Your child’s care team will be led by a pediatric cardiologist and pediatric cardiac nurse practitioner who specialize in treating children with pulmonary vein stenosis.
• Our team is constantly looking at new and better ways to treat pulmonary vein stenosis, including the innovative use of chemotherapy drugs.
• Our team works directly with our cardiac surgeons and cardiac catheterization specialists when surgery or balloon dilation (to open the narrowed blood vessel) and/or stenting (using wire tube to prop open the vein) is necessary.

We are currently conducting a study using the drugs Avastin® and/or Gleevec® to treat multi-vessel intraluminal pulmonary vein stenosis in children. The purpose of this study is to determine the effectiveness of using these two chemotherapy drugs (along or in conjunction, depending on the child’s specific disease) to control the abnormal growth of cells that cause the pulmonary vein blockage. This therapy, if effective, could reduce the need for catheterization and surgery—current treatments that provide only temporary relief.