My hope for this blog post is to get it to as many people as possible so that when someone googles or searches for PVS, this blog post will pop up for them to read. I ask you to please share this post with as many as you can, after you read it!!
What is Pulmonary Vein Stenosis? (PVS) Pulmonary vein stenosis is a rare and serious condition in which there is an obstruction (blockage) in the blood vessels that bring oxygen-rich blood from the lungs back to the heart. It can be isolated to a single pulmonary vein, but most often occurs in multiple veins simultaneously. Most people have 4 pulmonary veins but some people can have 5.
Here is a simple overview of the Pulmonary Veins:
In Pulmonary Vein Stenosis the veins grow extra tissue that clog up the vein openings, thus creating a stenosis/narrowing of the vein. This in turn causes blood to back up into the lungs and cause higher blood pressure in the lungs, called Pulmonary Hypertension. The narrowing needs to be opened up to relieve that pressure build up so the lungs and the heart do not have to work so hard to pump the blood through the pulmonary veins and out into the body.
A child can have pulmonary vein stenosis for many reasons. It could be connected to another heart defect, it can be acquired after having heart surgery for another defect, it can also be congenital/ideopathic. The latter just means that the child has no other heart defects and has pulmonary vein stenosis primarily. PVS is so rare and so unique to each child and that makes it hard to compare cases and treatments.
Treatments for PVS:
Stenting (bare metal or drug eluding) — The drug eluding stents have everolimus on them that helps the vein stay open and slows the production of tissue growth.
Sutureless surgical repair — (open-heart surgery to widen the narrowed veins and improve blood flow from the lungs back to the heart)
Avastin/Gleevac (chemo drugs that help keep the veins open)
The ballooning and stenting are usually short lived and temporary fixes. The veins do not like to be messed with, frequent heart catheterizations are needed to continue to keep the veins open and grwoing with the child. This is a complex condition and still has a grave outcome but there are a few hospitals that are inventive and think outside the box in treating PVS. Texas Childrens in Houston is treating our daughter and has done an amazing job, totally saved her life!! Dr. Justino is a genuinely caring doctor and he is not afraid to try things to save a childs life. Boston Childrens is another facility that has a PVS program. They tend to be a little more invasive and have a different approach than Texas Childrens but both places put thier blood, sweat, and tears into fighting this disease!! If your child has this diagnosis, please do your research and arm yourself with knowledge. There are options for this disease, you just have to be willing to go other places to get treatment. Most hospitals and doctors do not even know much about PVS, thus they do not know how to treat it. PVS to most doctors is considered a death sentence, please go to someone who deals with PVS and has treated many cases. You are your childs best advocate!! Knowledge is POWER!!!