Bicuspid Aortic Foundation View From Here May 2007

Strangers in Two Worlds ?

There are two worlds where those with bicuspid aortic valve disease might find themselves. Perhaps because of the subtle, almost deceptive nature of BAVD, those who have it do not fit well in either of them.

Strangers in the world of congenital heart disease?

There is a world for those with congenital heart conditions. It is defined by many complex problems that require life-saving surgery at an early age. These are hearts with problems so severe that without early intervention there is no hope. An example is Tetralogy of Fallot, which is actually four different abnormalities of the heart that are found together. Much of the pioneering heart surgery that began over 50 years ago was performed to help these infants and children. Surgical advancements have saved many of them, allowing them to reach adulthood. Their survival has resulted in an emerging specialty for them as adults with congenital heart disease.

In contrast with these complex cardiac conditions, the bicuspid aortic valve must seem like such a simple thing. Although it is the most common congenital heart defect, it may not cause any problems at all in childhood. Perhaps if BAV is found in combination with other defects, treatment may occur in this setting. But generally the BAV does not find a home here. I noticed this when I attended a large conference two years ago. The congenital heart disease agenda held nothing of interest for me - there was no focus on the bicuspid aortic valve!

Strangers in the world of valve disease?

There is another world, the world of heart valve disease. This is a world focused on problems with the valves of the heart - most often the aortic valve and the mitral valve. The failing BAV is found here, included along with the other reasons for heart valve issues. Many with BAV, like my husband, are first treated in this world, undergoing life-saving valve surgery. But the full picture of BAVD is not contained in the aortic valve with only two leaflets. Today we know that bicuspid aortic valves are associated with aortic aneurysms. We also know that there is an increased risk of brain aneurysms when compared to the general population. Some have connective tissue abnormalities expressed elsewhere in their body. And there are issues such as blood pressure that surgery alone does not solve. Yes, many aspects of BAVD make it a stranger in the world of valvular heart disease also.

The world of BAVD is not a simple one.

It was when my husband's aneurysm was discovered in 2001 that I began to understand there was much more to the BAV experience. We were shocked by the diagnosis of the aneurysm. I remember very clearly my husband's surgical consultation at that time. Would this surgeon understand bicuspid aortic valve disease? Yes, he not only understood the connection between aneurysm and BAV, but the blood pressure issues as well. He did what no one else had done - he immediately addressed my husband's blood pressure. Finding understanding and help in that surgeon's office was an unforgettable moment. That day we found a place where bicuspid aortic valve disease was appreciated. We found a world we were not sure existed. That surgeon was Dr. Sharo Raissi, whose vision resulted in the creation of the Bicuspid Aortic Foundation.

The complex nature of BAVD was reinforced again very strongly when I sat beside Carrie Mettler as Dr. Wouter Schievink told her about the aneurysm in her brain. What tremendous shock, but also consolation in the presence of a neurosurgeon who understood that BAVD may also be expressed in the blood vessels of the brain. Along with his compassion, this physician had the skill to save Carrie's life. Again that day, we were in a world where the complexities of BAVD are appreciated. We are very grateful for Dr. Schievink's participation on our Scientific Advisory Board.

Those with BAVD need a world focused on them;
a place where the variation and complexity of this condition are acknowledged.

I am encouraged by the increasing focus on BAVD as seen in the number of new references in the medical literature. However, premature loss of life due to BAVD continues. Recently I was told of a young husband and father with a known bicuspid aortic valve. He went to the local emergency department in distress and after many hours spent struggling to save him, his life was lost to aortic dissection. It is a tragedy that along with his valve, his aorta was not checked before it became an emergency. Apparently, he was a stranger in the world of valve disease. A stranger because it was not recognized that the aorta needed attention as well as the bicuspid aortic valve. It can be dangerous to have a BAV in the world of isolated valve disease. In that world, the diseased aorta that can tear and rupture is not recognized, especially in those so young and seemingly healthy.

And what of those like Doug Grieshop or Richard Houchin, whose first encounter with BAVD was expressed not by the valve, but the aorta? Is there a world where they are understood?

It is our goal that no one with BAVD or TAAD should find themselves a stranger. They should not doubt the reality of their own experience because they do not fit common perceptions about BAVD. Rather they should have access to all the life-saving information available today. One way in which the Foundation brings focus to BAVD and TAAD is through our September Awareness campaign. In addition to various volunteer efforts in their communities, again this year in September the Foundation will be hosting its second annual "Our Families, Our Friends" Conference in southern California. We are planning a variety of sessions and hope that many will be able to join us. Please check the website often for further details.

Together we are pioneers, not strangers,
creating a climate of hope.

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

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