BAV Aortic Aneurysm Research Break Through

Diseased Aorta - Enlarged and Twisted Shape

This week we have exciting news to share about BAV aortic aneurysm research.

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 

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

 

Continued Thoughts and Prayers for Adam

Adam was slated to have surgery last week.

Plans sometimes change due to circumstances beyond our control.

That is what happened to Adam.

He is now scheduled for surgery early Monday morning.

Our thoughts and prayers are with him, his family,  and his surgeon. 

The Three W's: What, Where, When

Coming to grips with having open heart surgery is a process, except in emergency situations. Adam had done this once before, when the aortic valve he was born with and the bulging aorta above it, needed surgery. That is how we at BAF first met Adam, as he researched information as part of his decision making process. 

This time, with infection in the picture, surgery needed to be done promptly to fully vanquish the bacteria and put a new, undamaged aortic valve in place. When was to be last week. Where was also settled - Adam lives in an area large enough to have not only an infectious disease specialty center, but also a skilled aortic surgical specialist. Peace with the What (mechanical, homograft, Ross procedure, bovine, or porcine for the new valve) also finally came after weighing the choices carefully.

Valve choice decisions, so many years after the first prosthetic valves were implanted decades ago, remain a thoughtful, individual decision based on many factors. As I heard many years ago now, there is no perfect choice! That is true, but there are many good ones.

When infection enters the picture, as it has for Adam, the considerations are altered. The risk of life-threatening infection returning and destroying the new valve weighs heavily, along with other factors, on the decision-making scale.

Ready for Surgery - Let's Get This Done!

With the help of his physicians and his own reading, Adam was at peace last week and more than ready very early the morning of surgery. By 6 AM he was prepped for surgery, partly sedated, and then . . . . he waited. And waited some more and some more . . . .

Finally, as morning faded and turned into early and then late afternoon, plans were abandoned for Adam to have surgery that day. His surgeon had been working to save someone else all those hours.

Adam says that whoever the other person was, they needed the surgery more than he did.

Adam's Turn - Monday Morning, June 10th

Adam will be ready once again, very early Monday morning. May it be his turn this time, since no one wants any complications to hurt him before the damaged valve with vegetation still clinging to it and any other remnants of infection are safely removed during surgery.

The plan is that Adam will have the Ross procedure. He has a skilled surgeon who will do all that is needed during surgery to clear the infection and save Adam in the best possible way.

Adam

Adam is a strong man, muscled and physically vigorous, with lots of energy! He is a loving husband and father, very much loved and needed. He is still young, with so much to accomplish.

How could it be that he has been so attacked by infection? He does not know how it got into his blood. Once finally diagnosed, he found that he was not alone, that there were so many at the infectious treatment center with their PICC lines in place, being treated for dangerous infections. 

Looking back, he says that, knowing what he knows now, with the first fevers he would have gone to the hospital and requested a blood culture be drawn. It is important for all with prosthetic or abnormal (bicuspid aortic) valves to realize that infection in the blood stream is a formidable enemy that does not play fair. The sooner it is found and treated, the better!

We join together in support of Adam and his family, 

asking that he have a safe, successful surgery, 

and that Adam will soon be home again, 

restored and infection free.

~Arlys Velebir

                           Bicuspid Aortic Foundation