BAV and Planning for the Big Picture

[Arlyss]

Sixteen years ago - getting closer to 17 now since he got sick from his very narrowed BAV! - my husband had his first surgery to remove his bicuspid aortic valve. We had no reason to think then that he would need further surgeries. We had very little information, other than his valve problem was from birth. Replacing it with a mechanical valve was the solution then, and life went on.....until the next surprise came along......

It is a different picture in 2006 for those with BAVs. More and more papers are appearing in the medical literature confirming that the BAV aorta is not normal. It is confirmed by studies in BAV children now that their aorta is enlarged when compared to children without aortic disease.

So, if my husband could start over today, with today's surgical capabilities and today's knowledge, our thinking and discussions would be very different than they were in 1990! We would be talking to surgeons about both the valve and the aorta - trying to decide if one surgery could/should handle both of them.

At the very least, if his ascending aorta was not replaced at the same time as his BAV, we would be watching it carefully to avoid dissection or rupture. And we would be aware that some day there could be another surgery because my husband might need his aorta replaced.

It does depend on which aspect of BAV forces surgery first - if both the valve and the aorta need surgery, it is quite straight forward. However, if the aorta is enlarged, a judgment call needs to be made about whether or not the BAV can be spared. If the BAV is the problem, what to do about the ascending aorta?

At least today patients and families should have enough information to know that these are the questions they face. It is not just the valve, not just the aorta, it is both that need to be evaluated and monitored for those with BAV.

In addition, I heard some information presented, not yet published, that indicates that those with BAV are about 8 to 9 times more prone to develop brain aneurysms compared to people in the general population. So the big picture includes being aware of that risk and being scanned for brain aneurysms also.

So while it is important to carefully understand everything possible about valve choices for BAVs, there is more to the picture and physicians must acknowledge this and help BAV patients, from childhood on, to have the very best lives possible, given what we know today. I hope all with BAVs find physicians who will work with them in this way.

Best wishes,
Arlyss

 

[aussigal]

Thankyou so very much Arlyss for all the hard work you have done to put together such a huge easily understood library of info for those of us new to BAVD.
I have learnt so much in this last year. I also had lots of scans (and a couple of surgeries) in order to try keep on-top of this and so-far so good. We know what is still normal and what is enlarged and have baseline figures and scans of the whole Aorta and even my brain. I would never have had any of this done if I hadnt come across your site (my Cardio didnt even think I needed a brain-scan but I insisted.). I would still be a walking time-bomb and our boys would also be time-bombs. I am confident that with this knowledge we will avoid many of the dramas that could have happened due to this dodgy gene we share.
I am curious as to how many others have gone ahead and had the brain and whole aorta scanned...and I also wonder if I should get the kids brains scanned as well...

 

[Arlyss]

Tonia,

You have done so well in saving your own life and being proactive with your children. I am so glad your doctors have been willing to work with you. No one has all the answers today, and it is important to have physicians who partner with us and learn from us!

There are more papers being published now regarding children, and there are some new references listed on the Medical References page of the Bicuspid Aortic Foundation at www.bicuspidfoundation.com

It is very understandable that on a forum like this there is a great deal of dialogue about the valve. The valve is extremely important, as my husband and I can attest to - my husband still fights to overcome the effect of that major stroke 10 months ago.

However, it appears that from many of the discussions here, physicians are still talking to those with BAVs only about the valve. BAVs need more than that, so that they can have the normal life span that they should have.....not lose their life because of abnormal blood vessels. I am thinking tonight of a man who lost his life last October......some years before he had the same mechanical valve put in him that my husband had. He went to the hospital with symptoms, and despite the family asking that everything possible be done to save him, he lost his life..... He should have been warned about his aorta, it should have been watched, and he should still be alive today....... I never met this man or his family, I only heard what happened, and I feel very very sad about it......

Best wishes,
Arlyss

 

[aussigal]

Thanks Arlyss...
I sometimes feel like I am over-reacting to all this BAV stuff but then I remember my Dad who died at 38 of what could well have been an aneurysm and his Dad who passed at 52 of a brain aneurysm. Hopefully I am helping to stop this happen again in our family. Those are the sort of surprises we dont need.