April 2007

When my husband was diagnosed with an ascending aortic aneurysm in 2001, we had no idea why his aorta was bulging dangerously above his mechanical valve.

I turned to the Internet then, searching for information, and found the
abstract of a paper by Dr. J. M. Burks, published in 1998. It described a relationship between bicuspid aortic valves and ascending aortic aneurysms, giving the examples of two young men. One of those young men had his bicuspid aortic valve replaced 10 years earlier. For my husband at that time, it was close to 11 years since his valve had been replaced.

Finding Dr. Burks' paper was the beginning of understanding why my husband had developed an aneurysm in his ascending aorta. It was also the beginning of my appreciation for the medical literature.

In 2005, the reason for my husband's extensive stroke was not obvious. When a TEE discovered fine hair-like strands coming off his mechanical valve, again I searched the literature. And again, there were papers. There were only a few, but they described these strands being present when strokes had occurred. This information made the decision to remove the mechanical valve so much easier.

Today I am very grateful to these authors, whose work sheds light on the path walked by those with BAVD and TAAD.

There are a number of newly published papers regarding BAVD and other forms of TAAD.  Following are links to a few of them.

From Yale, a paper about aneurysms in those whose BAV had not been replaced:

Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve

From Germany and the US, papers exploring the mystery of abnormal aortic tissue:

Endothelial nitric oxide synthase in bicuspid aortic valve disease

Expression of matrix metalloproteinases and endogenous inhibitors within ascending aortic aneurysms of patients with bicuspid or tricuspid aortic valves

Also from Germany, this paper describes a study of familial TAAD in three generations:


Actual management of patients with familial ascending aortic aneurysms and type-A aortic dissections


Dr. Randall Griepp published his ground breaking work on aortic arch surgery in December 1975,
Prosthetic replacement of the aortic arch.  In 2007, additional work on surgery of the aortic arch continues to be published. The Bicuspid Aortic Foundation is very grateful to Scientific Advisory Board members Dr. Randall Griepp, Dr. Teruhisa Kazui, Dr. Nicholas Kouchoukos and the other authors of the following papers:

Optimization of aortic arch replacement with a one-stage approach

Aortic arch replacement using selective cerebral perfusion 

Aortic arch replacement with a trifurcated graft 


So many with BAVD and other forms of TAAD are very athletic. There is a new paper on this topic from Washington University School of Medicine in St. Louis:

Approach to the athlete with thoracic aortic disease

Stents are now being used in the descending and thoracoabdominal aorta in some situations. Following is a paper from the University of Nebraska regarding these stents or "thoracic endovascualar aortic repair (TEVAR)".

Risk factors of neurologic deficit after thoracic aortic endografting

The following paper from the Texas Heart Institute discusses the outcome of treating thoracoabdominal aneurysms with open surgery over approximately 20 years. As the first sentence in the background section mentions, the newer endovascular (stent) approach to treatment must be evaluated by comparison to the established results of open surgery.

Open surgical repair of 2286 thoracoabdominal aneurysms


These papers are a sampling of the recent literature regarding BAVD and TAAD. It is a great encouragement to see papers such as these published. Many lives through out the world depend on this work.

Although we do not have all the answers today,
work continues on BAVD and TAAD ,
creating a climate of hope
.


                      
Sincerely,
Arlys Velebir
Chair, Board of Directors
Bicuspid Aortic Foundation



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New Papers on BAVD and TAAD
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