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

Unlocking the Mysteries - BAV and TAD September Awareness 2018

Still struggling with mysteries of the heart
  202 years since its invention,
 the stethoscope remains an important device
 for probing mysteries of the heart
   

"The practice of medicine is the struggle to unlock the mysteries within the body, to understand them in the hope of applying treatments to save life...."

This is my very own definition for the practice of medicine, reflective of the many years I have lived with struggling to understand and thwart BAV and TAD. I said this to a beloved cardiologist last year. He has been practicing for a very long time, and he did not disagree with me.

No one will ever convince me it is not a struggle. On the surface, what is known today was known 100 years ago. Do we have treatments now? Yes, but there is such a struggle to know what is happening, when and how to apply treatment, and will the treatment work . . . .?!

Too many times, there is no clear understanding. 

Too many times, there is suffering, injury, death. 

I was with one of our BAF board members recently for a consult with a new doctor. He remarked with astonishment that her medical records are a saga that rivals the novel War and Peace! So many different specialties, all searching for keys to understand her. We can only hope this particular specialist will be successful with his chapter of her story!

As September begins, I am looking forward to traveling to the University of Ottawa later this month, to visit the  Molecular Genetics and Cardiac Regeneration Laboratory . Dedicated to studying congenital heart defects, about half of their work is dedicated to unlocking the mysteries surrounding bicuspid aortic valves. They are co-located with the University's medical school and the Ottawa hospital. I am so grateful to be welcomed by them in our shared quest, our search for answers.

I invite you to share my visit to the University of Ottawa. Through this blog, I will have pictures, information, and stories to share. One thing I already know, I will be with those intensely focused on unlocking mysteries about the hearts and entire bodies of those born with BAV, and their trileaflet aortic valve family members too. I will be with those who understand the quest, the struggle, and are up for the challenge of this fight for life.

This September, 

there is no quest more worthy,

 no struggle more worthwhile,

 than seeking to unlock the mysteries

of BAV and TAD in our families,

Creating a Climate of Hope,

~ Arlys Velebir

Bicuspid Aortic Foundation