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| Diseased Aorta - Enlarged and Twisted Shape |
Desperate Need to Understand and Help
We are in desperate need to better detect, understand and appropriately help those with BAV and aortic aneurysms and their families.
Writing this, I remember Doug Grieshop, Forever 33, who left a young son and unborn daughter the day that his aorta ruptured. His BAV was never diagnosed, his aneurysm never found in life - only by autopsy.
Researchers Are Unmasking the Villains
At BAF we are very excited by published research, highlighted in the press this week, describing cellular villains in the aortic wall that destroy tissue around them and weaken the aortic wall.
This is work lead by Dr. Pickering, a research scientist, and Dr. Chu, an aortic and cardiac surgeon, who have collaborated to study aortic tissue removed from human patients. They have published their findings, reporting on the existence and trouble caused by abnormal smooth muscle cells in the wall of the aorta.
According to Dr. Pickering, these abnormal cells produce an enzyme that eats away at the tissue around them, destroying and weakening the aorta.
Following are press release and links with more information
Exchange Magazine
Robart's Research
Dr. Pickering's background.
Dr. Chu's background.
Research Paper: Seno-destructive smooth muscle cells in the ascending aorta of patients with bicuspid aortic valve disease
Light Breaking Through the Mysteries
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| Can Light Shed by Research Translate to Better Care? |
This give us tremendous hope, but there is much more to do.
We know from the work done at Dr. Mona Nemer's Lab on GATA about the genetic abnormalities and BAV in mice families, who are so very much like our human families.
There are studies of abnormal blood flow through BAVs, of aortic wall stiffness.
There are studies about the leaflet configuration abnormalities of BAVs.
There are many things to study!
Now we know something important about the aorta tissue - that these abnormal smooth muscle cell secretions are breaking down the aortic wall!
There is not just one thing, but many things, to think about! What else is happening, that we do not know today? No doubt there is more to understand.
Light is just beginning to shine on multiple factors, some combination of which is making each BAVer experience somewhat unique, but with common tendencies and themes.
Aneurysms and Abnormal Smooth Muscle Cells
If we can identify these smooth muscle cell villains, can we get rid of them before they cause trouble?
Can we find out what causes these smooth muscle cells to become abnormal and prevent that from happening?
The Challenge of BAV Misunderstandings
It is a very difficult challenge when someone is the picture of glowing health on the outside, vibrant and active, but with a serious vulnerability on the inside - prone to aortic valve failure, aneurysm, infections.
Anything that sheds light on what is wrong on the inside helps both patients and physicians believe the reality of the BAV experience.
In the future, if aortic aneurysm disease were better understood, it might help physicians think about and look for BAV and an aneurysm in someone young and healthy-appearing with symptoms, like Doug Greishop, in time to save them. He lived in a state with world-renowned aortic surgical capability. It is tragic that he did not know, that no one knew in time, that he needed that expertise.
While we rejoice over the research and wait for it to translate into better care, we are very grateful for every successful aortic surgery that saves someone born with BAV and their TAV family members! And we do not forget those we have lost.
Today, we see light breaking through, but BAVers remain vulnerable.
Someday, there will be more light than shadows in the experience of BAV families!
Answers are beginning to come,
Creating a Climate of Hope!
~ Arlys Velebir
Bicuspid Aortic Foundation
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