Two kinds of Bicuspid Aortic Valves: Functional and True

[drivetopless]

I had my first surgical consultation yesterday and learned something I did not know about BAV, so I thought I would share.

Assuming I understood the surgeon's explanation corrrectly here's a summary:
1. True BAV -- true 2 leaflet, symmetrical BAV. More often associated with Connective Tissue Disorders. If you have this type, my surgeon would recommend proactively treaing the aorta (replacement? or reinforcement?).
2. Functional BAV - more common. This is where two of the three leaflets have fused, forming an assymetrical BAV. If you have this, it is inconclusive if you have the associated connective tissue issues that lead to aortic anuerysms.

I have to have some test done as he can't tell from the echo which type I have. I had a TEE 12 years ago that might have been helpful, but those records have long been destroyed.

So, I'm researching this to learn more about types of BAV and find references to:
1. congenital vs aquired BAV
2. 3 Morphology types: type 1, fusion of right and left coronary cusp; type 2, right and non-coronary fusion; and type 3, left and non-coronary fusion. Type 1 BAV is associated with male gender and normal aortic shape but a larger sinus diameter. Type 2 leaflet morphology is associated with ascending aorta dilatation , larger arch dimensions and higher prevalence of myxomatous mitral valve disease.

My head is spinning. What do you guys know about this? I know that pathfinder posted some good info on this in 2008 under "BAV does NOT mean a systemic connective tissue disease"

References:
http://icvts.ctsnetjournals.org/cgi/content/full/10/4/535
http://circ.ahajournals.org/cgi/content/abstract/circulationaha;48/5/1140

http://heart.bmj.com/content/94/12/1634.abstract

 

[Ross]

2. Functional BAV - more common. This is where two of the three leaflets have fused, forming an assymetrical BAV. If you have this, it is inconclusive if you have the associated connective tissue issues that lead to aortic anuerysms.

That there isn't really BAV. That is simply a tricuspid with a fused leaflet.

I know, doesn't answer your question, but thought I'd throw that in just to drive you topless.

Ross is wrong on this one!

 

[drivetopless]

Hmmm. . . . . .What about these three web images-- BAV's have the "fused" look:

http://my.clevelandclinic.org/heart/disorders/congenital/congenvalve.aspx

http://www.google.com/imgres?imgurl...mage_result&resnum=3&ct=image&ved=0CA0Q9QEwAg

http://www.google.com/imgres?imgurl...mage_result&resnum=1&ct=image&ved=0CAkQ9QEwAA

 

[Ross]

Hmmm. . . . . .What about these two images-- BAV's have the "fused" look:

And again:

See, you can teach an old dog new tricks. Never saw one like that, so I guess I don't know a Bicuspid from a Tricuspid. Come hether and let me have a looky. I'll go back to my hole in the wall now.

 

[Maryka]

When (after 15 years of echos) Dr. Liang at Stanford finally found a bicuspid aortic valve for me, you could see that two leaflets were fused, their teeny-tiny points very close together. To my (admitedly non-medical eye) they obviously were formed that way from birth. Otherwise the leaflets would have to had to have been almost completely overlapping and glued together.

 

[skeptic49]

Having a murmur from birth (or detected at an early age as mine was) would indicate a congenital abnormality as does having a dilated aorta in conjunction with BAV.

Jim

 

[aussigal]

Here is my contribution to your confusion and learning....
I have/had the true bicuspid and the dodgy Aorta and the Connective Tissue disorder. I have 3 sons...2 of whioch are BAV owners...1 has a true BAV like me and also has a slightly enlarged Aorta, the second has the fused BAV and also has a mild coarctation. I can also add that as you produce heirs if you get 1 with a BAV you chance of repeating that increases greatly...my 1st born is the fised BAV son and my 3rd son is the true BAV...my middle son decided to be different and have MVP instead which has been suggested is the "poor cousin" to BAV...dont know why we/me was so lucky to produce 3 sons with heart issues but yeah...it happens...all my boys are fit and healthy and so far have suffered no troubles etc...
Arlys at the BAV website is brilliant and offered/helped me a lot when I was learning about this stuff.

 

[Lily]

Spinning head?

Yeah, and then even an accurate diagnosis isn't always possible because a valve might be so calicified that it's hard to tell what it ever was. Was it a "true" bicuspid, or a fused bicuspid, or some other type of malfunctioning aortic valve? The other thing in my signature, a coarctation of the aorta, is (I think) another telltale sign of a "true" bicuspid. And, in your research, have you discovered that those with bicuspid valves, maybe even various forms of bicuspid valves, tend to have clear arteries? How could that happen with all these different types, causes, and/or degrees of bicuspid valves -- if they're not related in some way to a certain disorder? I can't help but wonder -- and all I'm really doing is wondering here -- if a lot of this is just "degrees of severity" within the congenital or genetic flaw. And then consider that there may be more than one thing going wrong with the valve. It could have had some form of congenital flaw and then a person could have had endocarditis on top of it. And then, of course, there's the possibility of "connective tissue" problems; but they don't happen all the time -- or do they, but in their own sweet time, possibly later in life for some patients than others? I think all of those possible variables are a real reason why the longevity of any given replacement valve can be technically unpredictable.

BTW, I'm the only "known" bicuspid in my entire family even though there have been a few aortic valve repairs and replacements within the family. One of my near relatives seems to me to have some Marfan-like qualities but doesn't have a bicuspid valve either.

 

[tobagotwo]

The problem is that the rules immediately stretch and break in real life. Most people with true bicuspid valves never have to deal with a cardiologist for it, even for a valve replacement, much less for aneurysms. Some people with fused-type, semibicuspid valves do have conective tissue disease and go through a great deal of surgery for the valve and for expanding arteries. You just can't tell.

Having a TEE may or may not tell you which it is, but it's largely irrelevant to your situation, in terms of telling you whether you have connective tissue add-on issues. Looking for expanding arteries over time will be more telling. Unless you have a specific reason other than that to have the TEE, I would consider not having it. It's intrusive and unpleasant. Consider relegating it to those things you just do when you need to.

Some strictly personal trending indicates that the earlier in life the valve gives you trouble, the more likely you are to have other connective tissue problems. Also, the more other valve and arterial issues that present, the more likely you are to have CTD. Most people with BAV don't ever have to do anything about it. Of those that do, about half have followup arterial problems, ranging from a relatively simple root replacement to advanced surgeries to replace segments of the aortic arch or the abdominal aorta.

Arterial problems are not limited to the aorta, although that certainly is a prime roadway for aneurysms. More subtle, but equally dangerous: intestinal and intracranial blood vessels which grow more fragile with age. They can be monitored, if you're smart about it.

I wish you luck on your journey. May you find that you have no more than a leaky valve.

Best wishes,

 

[COLLEEN S]

If you are born with one leg, or if you are injured and have one missing, does it matter?

 

[drivetopless]

It matters if it is a determining factor in deciding the degree of pre-emptive repair of the aorta (connective tissue disorder) at the time of valve replacement.

I learned today my BAV is the "True" type and while I have no aneurysm today, I am on the more likely list for forming one as time passes..

 

[Julieaka]

Thanks Drivetopless - I think it does matter - I am a WOMAN no Marfans..... with a definite congenitial bi-cuspid valve and "discovered" an ascending anuerysm 5 years ago that has "hung" around 4 cm - how long it was there, we do not know. I am going to a surgeon today and will definitely discuss BAV-D and what else he may find when he gets in there. So far at a top cardiologist (and consulting with a couple of others) they won't talk BAV-D with me, but then they were also not recognizing symptoms that seem pretty clear when I get on this forum. I think it has alot to do with being a young female, no Marfans..... although now I have crossed the 40 mark! lol..... they just don't have data on patients like me and they just don't "know" . I truly appreciate the attempt to explain the valve size/shapes because this will help me.