Pulmonary Vein Stenosis Program | Research & Innovation

The Pulmonary Vein Stenosis Program at Boston Children’s Hospital bases treatment on the latest clinical research. Over the past 20 years, the team has been crafting an innovative strategy in its approach to pulmonary vein stenosis (PVS) based on a new way of thinking about this rare and unusual disease. The strategy involves treating the condition on several different levels.

Use of oncology drugs

The first breakthrough came in realizing that PVS is not like other types of congenital heart disease (CHD). While other types of CHD don’t grow back, PVS does. This realization led our team of doctors, researchers and surgeons to begin looking more closely at the substance that causes blockages in the pulmonary veins of children with PVS. They discovered it was similar to a type of cancer cell, called a desmoid tumor.

This led them to look at chemotherapy regimens as a new way of treating PVS. In 2009, the team moved forward with a single-arm trial using two drugs (imatinib mesylate and bevacizumab) that were already being used in pediatric oncology. The research program very quickly evolved into a clinical program, as PVS families started hearing about it and wanted another treatment option. So far, the approach seems to be working and the program continues to treat patients and get national referrals. The team published their results in The Journal of Pediatrics in 2018.

Aggressive follow-up

The program also uses aggressive follow-up to carefully monitor patients. The team’s dedicated nurse practitioner checks in with patients often to triage symptoms and they run more frequent tests. This allows clinicians to find — and treat — any problems early.

New techniques in catheterization and surgery

Over the years, the catherization team has used their extensive experience in treating PVS patients, as well as angiography and intravascular ultrasound, to modify their approach to PVS in the catheterization lab. This has allowed them to achieve better, more effective dilations with traditional equipment.

The surgical team has also taken a new approach to the treatment of PVS by looking at each vein’s specific anatomy. The surgeon can then choose the right intervention to improve the anatomy and geometry of that individual vein. In addition, the surgeons have discovered new ways of suturing the veins that has been effective in keeping them open.

Working together on these fronts, the team continues to find new ways to treat PVS and offer hope to children with even the most severe forms of PVS.