Monday, September 3, 2018

BAV Current Research - Is Anyone Trying to Understand Me?

Leonardo da Vinci's Drawing of BAV 



Thanks to the anatomy studies of Leonardo da Vinci, and the work of the great physicians Osler and Abbott, we know that BAV and the associated complications have plagued humanity for centuries. 

So where are we, in 2018, in terms of understanding BAVers and their families?



How Can We Truly Fix What We Don't Understand?

It still hurts when I remember how naively we repeated to ourselves and others what we were told in 1990 - that a BAV valve replacement was a "fix" for life! It was a "patch" perhaps, and it saved life at that time, but it was not a fix, which implies no further problems.

I Have BAV - Does Anyone Know Who I Am? 

Am I the one who will go through life relatively unscathed? Am I the one who will have my first surgery before the age of 20, my BAV leaking and my aorta bulging? Am I the one whose enlarged aorta won't grow for years and then abruptly balloon out in size? Will I develop volatile high blood pressure? Will my BAV calcify and narrow when in my 30's, my 40's, my 50's?

Tragically for some, who they are in terms of the impact of being born with BAV is known only after death, when an autopsy is performed.

I Have BAV - Is Anyone Trying to Understand Me?
Highlighting Researchers this September

Who are the researchers taking on the truly daunting challenges associated with understanding BAV individuals and families today? As part of September Awareness, this blog will highlight some of the more recent work.

Researchers in Japan are meticulously looking at multiple angles that are part of the mystery of aortic aneurysms in those with BAV and aortic valve stenosis. The first link is commentary by them about their work.

Bicuspid Aortic Valve-Associated Aortic Dilatation ― What Is the Mechanism of Bicuspid Aortopathy?  As they write in closing: "In summary, bicuspid aortopathy is a multifaceted heterogeneous disease with at least genetic and hemodynamic factors contributing (Figure).2,3 Although the usefulness of medical treatment for preventing bicuspid aortopathy (aortic aneurysm formation in BAV) is controversial,5 more vigorous basic and clinical research attempts like this6 can lead to the discovery of novel therapeutic interventions and optimum treatments."

Here is their  paper, which gives us a glimpse into the complexity they are exploring in seeking to understand the tissue of the BAV aorta in contrast to a trileaflet aortic valve aorta, and why the BAV aortic tissue may be fragile.

Activation of the AKT Pathway in the Ascending Aorta With Bicuspid Aortic Valve

Yes, BAV and aortic aneurysm research is challenging.
We appreciate those willing to take up the challenge, 
Creating a Climate of Hope,
~Arlys Velebir
Bicuspid Aortic Foundation









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