Pulmonary Vein Stenosis Program
Our innovative approach
Historically, doctors have had to rely on one of the following treatments to care for children with pulmonary vein stenosis:
- open-heart surgery to widen the narrowed veins and improve blood flow from the lungs back to the heart
- cardiac catheterization with balloon dilation to try to stretch the vessel(s) open
- lung transplantation to replace the lungs and the pulmonary veins
Response to surgery or catheterization is usually short-lived. Typically, within three to four weeks, the obstruction recurs, and repeating surgery only provides temporary fixes.
In some cases, lung transplantation may be an option, but this comes with major risks, including rejection of the transplanted lung and serious infection. Patients must also take immunosuppressant drugs for the remainder of his or her life to help reduce the chance of the body rejecting the new lungs.
Using these methods, many children with the most severe form of pulmonary vein stenosis do not survive past early childhood.
The Dana-Farber Cancer Institute and Boston Children's Hospital are conducting a research study in patients who have multi-vessel (greater than two vessels) pulmonary vein stenosis. The purpose of this study is to determine the effectiveness of using two standard chemotherapy drugs that target myofibroblasts in pulmonary veins and control the abnormal growth of these cells.
The study involves treatment with Avastin and Gleevec, two medications used to safely treat childhood tumors. Multi-vessel intraluminal pulmonary vein stenosis is not thought to be a tumor, but it does share a common feature with tumors: the unwanted growth of cells.
Gleevec is given daily by mouth, while Avastin is given intravenously once every two weeks. They both have low side-effects and have been well tolerated in infants, children and adults. The goal of treatment is to stabilize the unwanted cell growth to improve patients’ life expectancy and quality of life. The trial involves treatment for one year, and can be coordinated close to a patient's home area.