bicuspid aortic valve

[caterb85]

I have been reading things about bav is not an isolated thing but a degenerating disease of the aorta. Does anyone know from talking to card or surgon if there is any statistics on this issue? Also something about brain aneurysms from this.

 

[caterb85]

bav

bav

that was the dumb site that said all the stupid stuff about your aorta being defective ever if you have the aneurysm part replaced, the real kicker was when they said about some for berry aneurysm in your brain forming from your bav.

 

[aussigal]

Its a bit confusing at first to sort out the medical terms and figure out how they can say an aneurysm in your brain may be related to a valve defect.

BAV is one of the many "Connective Tissue Disorders". The tissue that makes up your aorta, heart valves, blood vessels , tendons and many other tissues in your body are all made out of this same type of tissue. BAV is the name they have given to one type of this particular defect that most commonly presents with patients having a Bicuspid Aortic Valve and weak Aortic tissue. Brain/Berry aneurysms have also been found in a significant number of BAV patients. It is thought to be caused by a defective Fibrilin gene and tests and research are ongoing to get more answers.


The BAV website has alot of links to statistics and medical papers on this disorder.

I hope I have helped a little.

 

[caterb85]

queastions

queastions

I was not asking random strangers for answers I was asking them what there surgeons or specialist might have told them since we do not have alot that specialize in bav in our area.

 

[KathyM]

All I can say here is...I have a Bicuspid arotic valve with stenosis. I also had a small brain anuersym. I had the anuersym coiled and stented and will now be having the valve replaced April 10. I asked my cardio about the connection and he said that the key word is there COULD be some connection. I don't know that they have proven it yet

Yesterday when I saw the Nurse practioner for pre-op she asked if I would be in a study that is being conducted. She said I was a good candidate for this study. I am healthy and somewhat young 52. They will take a small sample of the heart wall and study it and look at many different factors. Age, health, genes and ect. to learn more.

That is really all I can say on this... Have a BAV and Brain Annie....


xoxo
Just
KathyM

 

[Bill M]

New to the group, I have beening reading articles since i came home in March, I felt i should add, since many of your postings have helped me deal with everything associated with the surgery.

I have had multiple problems, at 25 they found I had a bi-cuspid heart valve and coarctaion of the aorta (reason took so long to find I was seeing a bad GP for high blood presure, great cardiologist found it first visit). I had the coarctation repaired by Dr.Elefteriades at Yale, leading aortic surgeon. They monitored me for 5-years and in January found a large aortic root aneuryism, and my valve was starting to leak more. In march I had a mechanical valve and ascending/root replaced. They have discovered genetic markers for connective tissue disorder and seem to be learning more by the day.

They don't seem to know the exact links to brian aneuryism but have seen it in patients with the disorder. Prior to surgery they sent me for a brain scan and beleive I will be doing it every 5-years, will find out next week.

There definitley is a direct link between bi-cuspid aortic valve and weak aorta.

 

[Arlyss]

Hi Bill M,

So glad that they scanned your brain also. If more centers begin to do this, it will lead to greater safety for bicuspids and with time, greater understanding of what is happening.

Although the major aortic centers are aware of the BAV-Aorta connection, the brain is not usually checked yet.

We can all contribute to the cause by speaking to the physicians we work with about having the brain checked also.

Best wishes,
Arlyss

 

[Leah]

Yes, and, but...

Yes, and, but...

[For all intents and purposes, this really is a continuation of the aneurysm thread as well.]

I am grateful to folks on this site who have flagged the possible connective tissue/aneurysm/etc. issues. Without these postings I would not necessarily have identified these issues, which now are the basis for further questions and investigation.

At the same time, I'm concerned that there's the potential for us lay people to take this stuff out of context and create our own medical reality that's a little or a lot off-base.

I had the privilege of talking on the phone the other day with one of the national-reputation docs touted on this website. (I called his office to scope out the possibility of a consult and got a call back from the doc, who took quite a bit of time with me.) In addition to briefing him on my history, I asked about his take on the connective tissue/brain aneurysm/aortic aneurysm issue. He seemed very much up to speed on the issues and research, and - at least in our brief conversation - seemed to me to present a reality that was somewhat more modest than what I read on the BAVD website. For example, he did not believe that the brain aneurysm connection was at all established; he did tell me that aortic aneurysms were about 10x more likely with BAV patients, but indicated that this brought the odds up from 1/10,000 to 1/1,000. (It makes sense to me that this site would have greater concentration of people with complications.) He also had some comments, which I'm still digesting, about the likelihood/unlikelihood of developing aorta problems if you weren't already presenting with aorta problems. He absolutely was on top of the whole issue of aorta repair, and the need to scope out the whole aorta, and he discussed what he does with aortas that are enlarged but don't truly have aneuryms. [I'm going into this detail to make it clear that this is not some local/general surgeon who lacks background on the issues.]

The bottom line was that I came away with a sense that there are finite risks and issues to evaluate but not that my body is a walking time-bomb because of the BAV. I also came away with a better sense of why no one was rushing to evaluate my brain or my aorta separate from the progressive evaluation of the valve and the timing of surgery.

For what it's worth...

Leah

 

[Leah]

Did he cite you any empirical evidence for his opinions? Any studies we can look at for ourselves? ... I would love to see a good empirical study that measures the incidence of aneurysms in people with BAV at 1/1000. Then I could stop worrying about a lot of people.

No, it's a fair question but that wasn't the nature of our conversation. He extended me the incredible courtesy of calling me on his cell phone from the airport about an hour after I called his office to scope out how they handle consults, and we were talking primarily about my "case" to check out whether it made sense for me to travel for another consult - so the conversation about aneurysms etc. was one small piece of our discussion. I didn't ask him to back up his assertion with studies.

The main point of my posting was a concern that it's difficult, as an intelligent, educated lay person but nevertheless a lay person, to know the signficance of isolated data points and that there's reason to be cautious with the info. Your diligence and thoroughness about all of this is an inspiration. And...I don't know whether or not crunching the primary sources will be the appropriate path for me. I continue to see the reading I do more as a starting point for questions from the guys [unfortunately, not very many gals ;>) ] who do this full time and overtime than as a basis for me literally to form medical judgments.

This is a work in progress, though, and I have a lot to learn before I make any decisions (hopefully will continue to have a little time before the decisions have to be made)!

Take care,
Leah

 

[upchurch131]

I heard that a bicuspid aortic valve occurs in approximately 2% to 3% of births. Symptoms of this disorder also seems to manifest itself in the 4th decade of life. This is EXACTLY what occurred in my own experience.

 

[Ross]

Well were still trying to figure me out. I was tricuspid and yet exhibit the bicuspid problems. What is the link?????

 

[Mary]

Well were still trying to figure me out. I was tricuspid and yet exhibit the bicuspid problems. What is the link?????

http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17676603&ordinalpos=12&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Tobagotwo has written that he had a tricuspid that calcified to such an extent that they were certain it was a bicuspid until they operated.
Perhaps your aneurysms are the result of the connective tissue disease that bicuspids exhibit, Ross. The research is still so recent, I think they will be investigating this for some time before they have a good handle on it.

 

[Geordie97]

So, with all this discussion, where does it leave someone like me who was diagnosed BAV in 1959, had a commisurotomy in 1967 and a valve replacement in 1975? I have had no further problems with the valve and it is not suspected in my current CHF, diagnosed in 2006.

 

[Magic8Ball]

So, with all this discussion, where does it leave someone like me who was diagnosed BAV in 1959, had a commisurotomy in 1967 and a valve replacement in 1975? I have had no further problems with the valve and it is not suspected in my current CHF, diagnosed in 2006.

Just means that someone should be keeping an eye on your ascending aorta.

Ask the next time you are in seeing your cardio if there are any records of it being monitored for size.

 

[ALCapshaw2]

So, with all this discussion, where does it leave someone like me who was diagnosed BAV in 1959, had a commisurotomy in 1967 and a valve replacement in 1975? I have had no further problems with the valve and it is not suspected in my current CHF, diagnosed in 2006.

The correlation between BAV and Connective Tissue Disorder(s) is NOT 100% so maybe you are one of the "Lucky Winners" of the BAV / CTD lottery!

Just to be safe, it would probably be wise to have a CT scan or MRI of your entire Aorta every few years just to be sure nothing is developing. It's worth having a discussion with your Cardio about next time you see him (or sooner if you are concerned).

'AL Capshaw'

 

[lcwhitney]

To say that a BAV is a degenerating disease is what my ex -husband would call a diaper question because it depends on what you mean by that statement.All of us BAV people are born defective when our aorta only forms 2 flaps on its valve instead of 3 so, we all start out behind from the time before we are born. This one simple fact also slightly increase our heart rate just enoughfor the speed of our own blood to begin to damage the valve at the end of the largest and most important artery in the human body.If you mean to say we start life slowly falling apart at our aorta then you are correct.

Lettitia

 

[Duff Man]

It might've been a fluke, but I get an echocardiogram every year and one time the pressure gradient was significantly less, meaning it probably became better. It's steadily risen since that echo, but it was definitely reading better pressures. At the time I was really strict on diet and exercise. Who knows...

 

[Robert]

The secret is to put the condition in the back of your mind, but not out of mind so to speak, and live a normal life like there's nothing wrong with you

Dont rule out infection hitting your BAV either as another risk but I'm sure you guys already know that. At 16 years old I didnt even know I had BAV as I was fit and healthy, long distance runner, very athletic playing sports etc but developed an infection on my toe which left untreated eventually developed into a blood infection which then led to bacterial endocarditis - so much for being young and healthy! By that stage it was too late, there was too much infection growing around the swirlpool due to aortic murmur and it was eating away at the tissue inside the heart. At first I was at home and felt ill and thought it was the flu. That was until I started experiencing loss of body movement and functions. I couldnt even control my bowel movements or stand up/dress myself and it was a very frightening thing - I can remember being of sound mind but I just couldnt control my body!!!. I was admitted into hospital and they said something about a part of the abcess broke free from the heart and in simple terms caused a blockage in the brain that required removal. At this stage I went on immediate antibiotics but it was too late. Was then rushed by Westpac Rescue Helicopter to Sydney for surgery on the brain on a Tuesday (had some large metal cage bolted to my head whilst they drilled out a hole in the back of the skull to suck out the blockage) and then 3 days later had an artificial aortic valve replacement which began my long history. Nowadays if I ever see anybody with an infected wound I treat it very serious and tell them to look after it! I wish I was diagnosed with BAV beforehand so I could have avoided half a lifetime of grief... knowing you have it is a lot better than not knowing you have it thats for sure lol

 

[Duff Man]

Well were still trying to figure me out. I was tricuspid and yet exhibit the bicuspid problems. What is the link?????

when did they find out you were tri-cuspid? Did they think you were bi-cuspid because of an echo you had?

 

[tobagotwo]

There are many people who have bicuspid valves who never require surgery for them. There are many people who have surgery for bicuspid valves who never need medical attention for any other heart issues.

It's smart to get checked for aneurystic possibilities, both in the aorta and brain, if you find that you're bicuspid. Any enlargement should be considered an area to be watched. If you have your valve replaced, if there is any enlargement present at all (including in the root), I believe it's intelligent to insist that it be taken care of at that time, as it will likely cause its own surgery later anyway. I'm a long-time proponent of this.

I also think that special care should be used before harvesting a pulmonary valve for a Ross procedure in a bicuspid patient. Its the single, largest, preventable failure point for Ross Procedures, by my reckoning. Ross surgeons for BAV patients should be or have with them someone who is highly versed in recognizing developing myxomatous tissue and connective tissue disorders.

However, by my reading, the odds are still in your favor that most BAVs won't wind up with the other manifestations in a way that requires treatment, even if they have their BAVs replaced. The biggest, easy-to-recognize risk factor seems to be how early in life your BAV becomes a surgical issue.

What I'm suggesting is that you should be smart about finding out, but that you shouldn't live in fear afterwards.

Note that mitral prolapse is also a myxomatous tissue problem, but seems less often tied in with aneurysms.

Best wishes,