Didn't Realize BAV is Considered a Disease of the Entire Aortic Root !

[Texanbarbie]

I thought having a congenital bicuspid aortic valve was simply a malformation, but from what I've been reading today, BAV is a disease of the entire aortic root and has a propensity for both valvular and aortic complications. I'm sure this is nothing new to most everyone on this forum, but for me, this was amazing information - - and probably something I should have heard from my cardiologist. Another good reason to find another one !
This is what I just read from the Cedars-Sinai website:

Aortic valve malformation is the most recognized aspect of a congenital condition now known to potentially affect not only the aortic valve, but also the tissue of the aorta and other heart structures and organs. Bicuspid aortic disease (BAD) refers to the condition in its entirety, including the existence of some combination of the following:
Bicuspid aortic valve (BAV)
Aortic dilation, aneurysm or dissection (most commonly involving ascending aorta)
Other miscellaneous degenerative affects
Labile hypertension (rapid change with activity or stress)

The Aorta, Heart and Other Organs
Other distinctive features of bicuspid aortic disease (BAD) may be seen in the heart, where the mitral valve may also be regurgitant as a result of myxomatous changes.

The tissue of the aortic wall may be abnormal due to a degenerative process in the medial layer of the aorta. This results in a weakened, thin aorta that is unable to stretch and contract properly. The aortic tissue is prone to aneurysm formation and/or dissection.

In BAD, this is particularly true of the ascending aorta with extension into the arch. Medical treatment and elective surgery are important in avoiding serious, life-threatening events. Signs of degenerative disease may exist in other blood vessels and organs. Examples of this are subclavian or renal artery aneurysms, cysts in the kidneys and liver, hiatal hernias and effects on the eyes resulting in poor vision.

Bicuspid aortic disease is a condition that exists at birth. There can be significant variation in the way individuals are affected. However, the aortic root and ascending aorta are the aortic segments most commonly involved, and they are generally larger when compared to those in similar patients with normal trileaflet aortic valves. It is also known that development of aortic aneurysm or dissection may occur whether or not the bicuspid aortic valve has been replaced. Although it is possible to live a normal life span without experiencing problems due to this condition, many bicuspid disease individuals will require treatment during their lifetime.

SO GLAD I discovered this forum which opened my eyes and has given me so much insight !!

Barbara

 

[cldlhd]

Haven't heard about degenerative conditions in other organs or renal artery aneurysms but I guess there's all kinds of interesting possibilities. I had the root, ascending and hemi arch aneurysm. I had all that replaced with a graft,actually two, and my valve was repaired. Would be nice if they knew the specific gene(s) that cause it and there was a test for it. I'd like to know if my son has that active gene or not. Maybe he does automatically or maybe it can skip a generation. Perhaps there's someone out here less ignorant than me when it comes to genetics.
I don't like the term 'disease', makes me think of something that progresses and spreads. I prefer to think I had a plumbing issue that was fixed, maybe that's me being intentionally ignorant but they say it's bliss....

 

[Agian]

This may be wishful thinking on my part, but I'm not convinced of some of the things listed on the Cedars-Sinai website.
There have been hundreds of BAV folks on this forum, and these conditions have not been mentioned. If they were significant, you would think people would have said something, or relayed some information given to them.
Kidney and liver cysts are common and usually harmless.
"effects on the eyes resulting in poor vision" could mean anything from short-sightedness to near blindness.

 

[Paleowoman]

I only had bicuspid aortic valve, nothing else. Aorta checked in all echos.

 

[epstns]

I was told prior to surgery that BAV patients often have complications related to aneurysms in their aorta or other major arteries. My surgeon carefully reviewed scans of my aorta and abdominal arteries prior to scheduling surgery for the valve alone. It seemed that they looked, and if nothing was found that was out of the ordinary, it became just another valve replacement.

 

[cldlhd]

There seems to be professional disagreement about this, at least what I've experienced anecdotally. My cardiologist wanted to put me on a beta blocker even though my blood pressure is fine - like 117/72- because she says people with BAV are more likely to get aneurysms in other arteries but my surgeon who specializes in thoracic surgery and BAV repair says that the likelihood for aneurysm is just from the aortic root up to the beginning of the arch.

 

[pellicle]

There seems to be professional disagreement about rhis, ...t my surgeon who specializes in thoracic surgery and BAV repair says that the likelihood for aneurysm is just from the aortic root up to the beginning of the arch.

FWIW I asked my guy the same thing and got the same answer.

 

[cldlhd]

FWIW I asked my guy the same thing and got the same answer.

Well that's good to hear, I wonder why the difference of opinion other than the fact that people like to disagree. Maybe there's been people who have had BAV who also have some other kind of issue that causes problems in other areas

 

[Paleowoman]

Well that's good to hear, I wonder why the difference of opinion other than the fact that people like to disagree. Maybe there's been people who have big BAV who also have some other kind of issue that causes problems in other areas

People with Marfan's syndrome have bicuspid aortic valve, aaortic aneurysm and dissection along with all sorts of other connective tiisue disorders. Have a look at the list of problems they can get: http://www.marfan.org/about/related-disorders

 

[MethodAir]

There seems to be professional disagreement about rhis, at what I've experienced anecdotally. My cardiologist wanted to put me on a beta blocker even though my blood pressure is fine - like 117/72- because she says people with BAV are more likely to get aneurysms in other arteries but my surgeon who specializes in thoracic surgery and BAV repair says that the likelihood for aneurysm is just from the aortic root up to the beginning of the arch.

From what I've been told by an unbiased member of the medical community, a lot of cardiology (and its focus on neurohormones and drug treatments) is not based on science. The medical community is too fond of labels, "failure this, disease that"...creating fear and dependence, treating the symptoms and not the root of the problem. Well, they create an industry out of this stuff.

It makes sense that tissue close to a malfunctioning valve might be prone to greater wear and tear, especially when engaging in very high intensity activities, and that surgery (in many cases) addresses the root of the problem.

 

[cldlhd]

Thanks for the link.

 

[Fundy]

Well that's good to hear, I wonder why the difference of opinion other than the fact that people like to disagree. Maybe there's been people who have had BAV who also have some other kind of issue that causes problems in other areas

After seeing a study (http://www.ncbi.nlm.nih.gov/pubmed/20439844) suggesting strong link of brain aneurysm to BAV, I asked cardiologist about the need for a possible brain scan.

Her response was basically that as far as what's accepted and known in that type of correlation; was that coarctation of the aorta is sometimes correlated with those with BAV and that those people having coarctation of the aorta there is a higher risk of brain aneurysm occurring.

So someone with BAV having CofA is at higher risk of brain aneurysm, but someone with just BAV and no CofA is at no more risk than the general population for having a brain aneurysm.

The only other concern she had ever mentioned with me that would need to be watched, was of the aortic root and ascending aorta, as they seem to happen often in correlation.

 

[cldlhd]

After seeing a study (http://www.ncbi.nlm.nih.gov/pubmed/20439844) suggesting strong link of brain aneurysm to BAV, I asked cardiologist about the need for a possible brain scan.

Her response was basically that as far as what's accepted and known in that type of correlation; was that coarctation of the aorta is sometimes correlated with those with BAV and that those people having coarctation of the aorta there is a higher risk of brain aneurysm occurring.

So someone with BAV having CofA is at higher risk of brain aneurysm, but someone with just BAV and no CofA is at no more risk than the general population for having a brain aneurysm.

The only other concern she had ever mentioned with me that would need to be watched, was of the aortic root and ascending aorta, as they seem to happen often in correlation.

My aorta, just where it makes the turn to descend, has " mild kinking with no evidence of coarctation" according to the report. I can clearly see the kink looking at the image and if I remember correctly I asked my surgeon about it and he said it was fine. I'll probably ask him again when I see him in February-I really should tape those conversations on my phone for reference.
I can see where MethodAir is coming from . Sometimes it does seem like a lot of what you're told is based on what became "common knowledge" for whatever reason and it's rarely questioned and when contradictory information comes to light it's resisted. It's almost like a religion in some respects.

 

[AZ Don]

Fwiw, I've seen a study that contradicts the one indicating a connection between bav and brain aneurysms. I'll look for a link later and post if I can find it. I've found mixed evidence/opinions for anything more than the connection between bav and aneurysm of the root and ascending aorta.

 

[AZ Don]

Having trouble finding the study I mentioned. It may have been this one: http://www.ajnr.org/content/33/6/1182.full, which states:

There was no association between ascending aortopathy, bicuspid aortic valves, and IAs.

Where IA = Intracranial Anuerysm. But the study was limited to those with Coractation of the aorta.
This study looks more relevant: http://www.neurology.org/content/ea...by=yes&legid=neurology;WNL.0000000000001104v1
Out of over 1000 patients treated for IA,

317 had adequate echocardiography for assessment of BAV ... Conclusions: Our data demonstrate an association between IA and TAA, but not independently for BAV.

So that's not much better for those of us with both TAA and BAV. Strange that this study found no link to BAV. It's worth noting that the study that did find a link between BAV and Brain aneurysms included only 61 people with BAV, and found only 6 with Brain Aneurysms.

 

[pellicle]

Hey Don
Thanks for the links

I reckon that reckon that part of the reason you can never find them again is that our memory of what was written was influenced by the components we were interested in. I found that during the lit review of my Masters and spent many hours going through my PDF files trying to find what I swear I read the week before.

( often to find it in a different category

Getting data to answer our questions seems hard because our questions are from outside the typical research box. Us BAV folks have different interests and driving motivations than other researchers (who may be more generally specialised in aneurysms than linkages horizontally across "conditions")

Its classical that research only commences once y have submitted for your first aneurysm and the "healthy" population is unstudied.

Eg a previously healthy sportsman dies
http://mobile.news.com.au/sport/cri...cident-explained/story-fndpt0dy-1227135469050

 

[cldlhd]

I admit that I'm pretty much pulling this theory out of my sphincter but I was wondering if the coarctation of the aorta could lead to higher blood pressure in the brain and that could play a role in brain aneurysm.. Assuming the coarctation occurs downstream of where the arteries connect to the brain but upstream from where the arms tap in and they take your blood pressure from your arm. A kink in a hose would increase pressure before the kink but reduce it after. Could this be part of the reason brain aneurysms are more likely for people with BAV and coarctation? If this sounds ridiculous forgive me but I had to run over to my wife's work and fix her car ( alternator ) after I got done work and it's been a long one.

 

[pellicle]

Hi

.... Could this be part of the reason brain aneurysms are more likely for people with BAV and coarctation? If this sounds ridiculous forgive me but I had to run over to my wife's work and fix her car ( alternator ) after I got done work and it's been a long one.

to my reading of history some of the best advances in science come from people making seemingly wild assumptions ... so its all good IMO. Seems a valid research question.

 

[Glen Swan]

I admit that I'm pretty much pulling this theory out of my sphincter but I was wondering if the coarctation of the aorta could lead to higher blood pressure in the brain and that could play a role in brain aneurysm.. Assuming the coarctation occurs downstream of where the arteries connect to the brain but upstream from where the arms tap in and they take your blood pressure from your arm. A kink in a hose would increase pressure before the kink but reduce it after. Could this be part of the reason brain aneurysms are more likely for people with BAV and coarctation? If this sounds ridiculous forgive me but I had to run over to my wife's work and fix her car ( alternator ) after I got done work and it's been a long one.

My son was born with BaV and CoA (Coarctation of the Aorta). He had his CoA fixed by simply removing the narrow part and piecing the good ends together. After the surgery, it could renarrow after a certain period of time, but never did. He is all clear from that aspect. He is going on 4 years old this January and doing well.

That being said, he still has the BaV, which as we mostly know, is not engineered well enough and could cause complications later in life. Likewise, the CoA can still lead to higher blood pressure later in life even after a fix has been successful. For those reasons, in the event of complications, high blood pressure can occur, artery walls can weaken and aneurysms increase.

I personally believe, just based on the many vague documents and conversations I've had, is that CoA with BaV patients generally have a higher chance of hypertension. Thus, they have a higher chance of aneurysms due to the increase blood pressure and the fact that their arteries may be weaker than those without defects. It just so happens that one of those locations that happen in 10% of the patients is in the brain, but can form in other locations too.

Either way, I know from my end, my son will get checked up once a year for the remainder of his life. That's going to increase his and his doctors awareness about any issues that may crop up.

 

[cldlhd]

My son was born with BaV and CoA (Coarctation of the Aorta). He had his CoA fixed by simply removing the narrow part and piecing the good ends together. After the surgery, it could renarrow after a certain period of time, but never did. He is all clear from that aspect. He is going on 4 years old this January and doing well.

That being said, he still has the BaV, which as we mostly know, is not engineered well enough and could cause complications later in life. Likewise, the CoA can still lead to higher blood pressure later in life even after a fix has been successful. For those reasons, in the event of complications, high blood pressure can occur, artery walls can weaken and aneurysms increase.

I personally believe, just based on the many vague documents and conversations I've had, is that CoA with BaV patients generally have a higher chance of hypertension. Thus, they have a higher chance of aneurysms due to the increase blood pressure and the fact that their arteries may be weaker than those without defects. It just so happens that one of those locations that happen in 10% of the patients is in the brain, but can form in other locations too.

Either way, I know from my end, my son will get checked up once a year for the remainder of his life. That's going to increase his and his doctors awareness about any issues that may crop up.

I'm glad to hear your son's surgery came out well. I can't imagine how stressful that would be. My son has a trileaflet valve but I still want to push his pediatrician to get a full scan of his aorta.
Looking at the image of my aorta I can clearly see some kinking where it turns downward but I've never had high blood pressure or hypertension and the report said no evidence of coarctation. I figured that section could be removed or replaced with a graft so if it's something that will need attention one day I wish they would have done it when I had OHS back in Feb.
As for blood pressure and aneurysms I can't help but think back to when I used to do a lot of weight lifting and the veins would be bulging out of my arms. I always thought that was a good thing,big open veins but knowing what I know now about my aortic aneurysm I'd rather not think about it.. Exercise raises blood pressure but I guess since it's temporary the idea is that the rest of the day its lower than it otherwise would be so it's beneficial? Or maybe certain types of exercise for people who MIGHT be prone to aneurysm is a bad idea?
Thanks for the info.