aneurysm

[caterb85]

Does anyone know if having a asending aorta aneurysum due to a bicuspid valve makes you at a higher risk for developing other aneurysms.
we asked our Dr. but he said he didn't know. If so what other test should we have?

[Susan BAV]

I'm not sure, but I think so, because several years ago my cardiologist told me that, because I was born with the bicuspid valve, I was much more likely to also have a brain aneurysm.

[edit later - At that time I opted not to have a brain scan but needed one a few years later and was found to be aneurysm-free.]

[tobagotwo]

Because aneurysms are often caused by genetic connective tissue problems, the chances of someone with an aortic aneurysm having or developing another aneurysm (or an extension of that aneurysm) are higher than they would be for someone who hasn't ever had an aneurysm.

But it's far from a certainty. And the older you are when that first aneurysm shows up, the less likely you are to have another.

If you have or have had an aortic aneurysm, you should consider having yourself checked to determine if there might be any other vessel expansions. Once you have a baseline, you might consider redoing the checks on some type of a reasonable schedule, like biannually, or even every five years, if your insurance or means will allow it.

Best wishes,

[aussigal]

Yes...Bob is right...have a read of the BAV website and you will find more info on this...It is wise for us to have all vulnerable areas monitored....I bet you dont want to die of a brain aneurysm after surving OHS. You may recognise symptoms of other family members as well...a good time to suggest they get screened as well maybe?...

[KAJ]

After reading about this very issue, I asked my primary care doctor about it. He agreed it would be prudent to have an echo on myabdominal aorta. Fortunately the insurance company agreed with it. We now have a baseline.

Karl

[caterb85]

Does anyone know why alot of bicuspid valve people also get an aneurysm in there ascending aorta?

[Rich]

There was an excellent article about this subject posted quite some time ago.
Hopefully someone will find it and pass it along to you.
Rich

[ALCapshaw2]

Connective Tissue Disease (or is it Disorder?) often accompanies BiCuspid Aortic Valves. This can result in a 'weakness' of the ascending aorta which results in an aneurism.

There has been considerable discussion of this effect in the Forums.

'AL Capshaw'

[aussigal]

I dont remember having seen an article about this but I do remember what my Surgeon and Cardio discussed with me and showed me. I hope you can follow my explanation.

Due to the design of a BAV its opening is a different shape to that of a regular tri-cuspid valve (there is an excellent diagram of this on the BAV website). Its like a cats eye instead of almost round. This leads to the blood exiting the heart at a strange angle.
Picture this, squeeze the end of a hose or hold your thumb over it and it forces the flow of water to change., its very similar with a BAV. In my echo scans you can clearly see the blood shooting out at this weird angle and hitting the wall of the Ascending Aorta , this is the area where my Aneurysm was. Now add in dodgy connective tissue and you get an Aorta that eventually balloons out and then bursts in that area as it doesnt have the elastic properties it should have and is under increased pressure in one area.

I am not positive this is how or why it happens to all of us who get Aneurysms but it was the reason mine developed where it did.

[Philip B]

My surgeon simply expalined it as the same defective tissue which is found in the bicuspid valve extends up into the aorta. Often the devective tissue in the aorta is not smooth inside and turbulent blood flow from the bicuspid valve stresses the defective aortic tissue. He further explained that he expected to find cysts and potmarked areas inside defective tissue removed from my aortic aneurysm.

It may be more complicated than his explanation, but at the time he offered this explanation, I needed a simple answer to the same question you asked.

-Philip

[Lynlw]

Here's some info on BAVD and aneurysms http://bicuspidfoundation.com/Bicuspid_Aortic_Valve_Disease.html#Anchor12

[caterb85]

I got on the bicuspid aortic foundation site and found it very frightening that a bicuspid valve can mean that your whole aorta is faughty. Has anyone else with this talked to there dr about the possibility that your aorta and brain should be checked on a regular basis? My husbands dr said he had a bicuspid valve but not a connective tissue disease like they tell you on this web site, who is right?

[Leah]

I had a rather strong reaction to the BAVD website info, like, maybe I've been on another planet in the 4 years since my diagnosis with a BAV and AS: In addition to coming to grips with the whole open-heart buzz-saw, do I have another major syndrome that no one has told me about? This includes a local well-educated cardio, a local well-educated surgeon, and a cardiologist at Cleveland Clinic who's facilitating my consult there in May (we've spoken a couple of times on the phone).

The overall message I've gotten (perhaps simplified) has been that, once the BAV/AS is symptomatic (so far not), it becomes seriously life threatening but that the replacement surgery is essentially the fix (allowing for complications, re-surgery if I choose a tissue valve and live to a ripe age, the need for continued monitoring, etc.). No one has mentioned the likelihood of a whole-body connective tissue disease, imminent threat of aneurism, etc. Also, the BAVD website suggests that there's a special protocol concerning blood pressure for people with BAVs. I usually have "normal" blood pressure (most often 120s over 70s or 80s, occasionally 130s, a couple of 140s in the last year). Certainly my BP is being watched but no one has suggested that we should get it down to some ultra-low point.

If I were prone to self-diagnosis (usually not but the BAVD site got my goat) I would worry about the fact that I've been nearsighted since 2nd grade (so is everyone in my family) and have had non-serious palpitations over the years (which haven't borne fruit in the course of some very extensive monitoring). (The site mentions these as additional manifestations of the syndrome.)

I will definitely follow up with my Dr. and also try to read more about this. But right now I'm trying to get a sense of whether this is established medicine, fringe medicine, cutting edge, something in between, or something altogether different. Also, if connective tissue diseases are associated with BAVs, is it all BAVs or is this a subset? I want to make sure I get all the information that's relevant to my situation, but also find it suspicious that in four years of trying to educate myself about this condition, I haven't found any references to this on any of the mainstream websites like Medscape, Cleveland, Mayo, AHA, etc

Comments? Help?

Leah

[Susan BAV]

This is my opinion.

Apparently there can be degrees of the bicuspid "disorder," and possibly different facets. Some people never need anything done to their bicuspid valves at all and are never even diagnosed until their death at an ancient age and upon their autopsy. Then some other people need surgery as infants, to repair their bicuspid valves.

Does the severity of the valve malformation indicate severity of any possible connective tissue problem?
I don't know if anyone knows.

I don't think any expert is exactly positive yet about how correct the different thinkings are related to those with bicuspid valves, whether one is correct or another is correct or whether there is a measure of truth to both theories depending on the degree of the problem within each patient--does the misfiring/malfunctioning valve cause aortic enlargement problems or is there always the possibility of connective tissue fragility in connection with the bicuspid defect...

[edit - an example: "The autosomal dominant inheritance of bicommissural aortic valve (BAV) (Online Mendelian Inheritance in Man #109730) in some families is well-documented; however, the inheritance of BAV with thoracic aortic aneurysm (TAA) is less clear. Whether the aneurysm is secondary to hemodynamic perturbation related to the valve abnormality or a primary manifestation of the disorder remains controversial. Guidelines are needed regarding the follow-up and treatment of these patients and their families. Thirteen families with at least one individual with TAA and BAV (BAV/TAA) were evaluated prospectively by standard echocardiographic methods or clinical history. Affected status was determined by the presence of BAV or TAA or a history of dissection, rupture, or surgical repair. Six of 13 families had at least two family members with both BAV and TAA, often in successive generations. Informatively, all 13 families had at least one family member with TAA in the absence of BAV. Thirty-five percent (39/110) of family members had BAV/TAA or TAA, and the majority of families (11/13) had maximal dilatation above the sinotubular junction (STJ). Vascular dissection or rupture occurred in seven of 13 families and in individuals with structurally normal aortic valves. Two families had non-manifesting, obligate carriers. Three families have members with other left heart outflow tract anomalies. This study confirms autosomal dominant inheritance with incomplete penetrance for BAV/TAA in these families. Furthermore, our data suggest that the component features, BAV and TAA, are independent manifestations of a single gene defect. To avoid the risk of early death, it is essential that all first-degree relatives receive echocardiographic follow-up at regular intervals regardless of the presence or absence of a BAV. This assessment must include imaging of the aortic region above the STJ."
http://www.ncbi.nlm.nih.gov/sites/en...ubmed_RVDocSum ]

Statistically, however, aneurysms can occur with many bicuspids; so it's a very serious aspect to consider.

I find it also very interesting that people with bicuspid valves usually have very clear arteries. Why? Does that indicate some possible slickness of the tissues? But could that also equate with weak tissues? Does anyone know yet?

I've read that men have bicuspids more than women at a ratio of either 3 or 4 to one. I don't know if that statistic has changed with the times because there are many women on this site who had bicuspid valves. If that statistic has changed, is it a true change in that more women have bicuspids than previously, or are just more women manifesting problems and needing their valves replaced. If the latter is true, why?

I'm a little more rare, perhaps, because I also had a coarctation of the aorta, which occurs with bicuspid valves. But most of the bicuspid people here have never been diagnosed with such a thing. I was told, as a young person, that the coarctation made me particularly vulnerable to sudden death. Is that why there aren't many here with that diagnosis? Did they not survive? I once read that the average life expectancy with an unrepaired aortic coarctation was 15 years.

So, there are so many possible variables, it makes my head swim.

I haven't visited that bicuspid website recently. When I have visited it, I thought there was some very informative information. But I also thought there were some theories presented also which may or may not be eventually proven.

Try not to worry, caterb85 [edit - and Leah]. Personally, I tried to find the best surgeons I could and I tried to make the best decisions I could and then I had to make peace with it, knowing that I'd made the best possible decision I could with the current knowledge available to me and the experts.

Best wishes :) .

[caterb85]

Thanks so much for your info and time to reply to my question. I wish you all th e best and hope all goes well.

[Arlyss]

Yes, it is shocking to learn the bigger picture about BAVD.

I was shocked when a large aneurysm was found in my husband's chest. No one was looking for it. We were told 11 years earlier that he was fixed for life when his BAV was replaced with a mechanical valve.

I was shocked when I heard the neurosurgeon tell my friend that she had an aneurysm in her brain, behind her left eye. We already knew about the BAV and aortic aneurysm in her chest.

And I was shocked when someone told me about the successful fight to save her husband's eyes, only to later lose the fight for his life in an ER. No one associated the eye problem with anything else. Yes, he had a known problem with his aortic valve. But when he went to the ER with chest pain and a heart attack was ruled out, they said it was anxiety. He died in that ER from a torn aortic aneurysm. Later, his wife found the words "probable bicuspid aortic valve" in the records kept by the cardiologist.

Knowledge has been slow to spread, but there is much more information now in the medical literature.

Here is a new paper, this month, from Stanford, in Northern California:

http://www.ncbi.nlm.nih.gov/pubmed/18374778?ordinalpos=4&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsP anel.Pubmed_RVDocSum

The phrase "connective tissue disorder" is used in the abstract.

I too have felt that we were on a different planet. I understand.

Today, with my husband and his family, we have arrived in a place where we know what is known, and we recognize that not everything is known.

We also know that we will never let down our guard. In the mean time, we are still together, despite BAVD.

Best wishes,
Arlyss

[Arlyss]

From a paper at the International Stroke Conference in February, 2008:

"Results: Intracranial aneurysms were found in 11% (5/47) of patients with bicuspid aortic valve and in 1% (3/288) of controls (p<0.01). Conclusion: Patients with bicuspid aortic valve are at an increased risk of developing intracranial aneurysms."

My husband and his family were scanned for brain aneurysms. They do not have any at this time.

But my friend did. How I and so many others would miss her, if it had not been found and "disarmed" in time.

Best wishes,
Arlyss

[KathyM]

I had a brain anuersym. In Nov 07 I had it a stent and 10 coils placed in it through the groin.

I also have BAV - stenosis. This will be replaced this Thursday.


Just
Kathy M.

[Arlyss]

Hi KathyM,

I am so glad that your brain aneurysm was found. You have my very best
wishes for your surgery this week.

Arlyss

[Leah]

Well, I obviously have quite a bit of reading to do - and need to be asking some serious questions once I've digested the info a little more. This came as a shock, and I won't know what to make of it all until I follow up, but I appreciate the resources very much.
Thank you,
Leah

[Leah]

[QUOTE=Arlyss]From a paper at the International Stroke Conference in February, 2008:

"Results: Intracranial aneurysms were found in 11% (5/47) of patients with bicuspid aortic valve and in 1% (3/288) of controls (p<0.01). Conclusion: Patients with bicuspid aortic valve are at an increased risk of developing intracranial aneurysms."

Arlyss, do you have a link to that study?

Thank you,

Leah

[MrP]

At age 49, I too was surprised to learn that I had an ascending aortic aneursym and after knowing of my heart murmur and BAV at the age of 10. I think aortic aneursyms are more common in those BAV patients with regurgitation vs. aortic stenosis; however, like me, many had both.
With respect to brain aneurysms and the study cited, a sample size of 47 BAV patients is small, and although I don't doubt increased risks in BAV, I think the margin of error in this study is rather large....something like the inverse of square root of 47.

[KathyM]

Thank you so much for the good wishes. I really appreciate it.

The brain surgery went very well and was as easy as a angiogram. I will have to have a MRA next month to check on the coils. I hope the valve surgery goes as well. I believe it will and I have so much to thank everyone on this forum for. I feel I am going in with such a huge amount of knowledge, all of which I have learned here. I had a friend of mine who's mother had the surgery 2 1/2 weeks ago. I was telling her some of the things on the posts and what I had learned. When some of these things would pop up for her mom she would remember what I told her and it helped the both of them. So you see how much this forum so many people.

Thank all so much, I will keep you all posted on my surgery.


xoxo
Just
KathyM

[Leah]

Thank all so much, I will keep you all posted on my surgery.

May your strong spirit carry you through!

Leah

[lcwhitney]

If I understood the cardiologist and neurologists correctly AN ANEURSYM IS A WEAKNESS IN AN ARTERY. This weakness only shows up if the conditions in your body create the correct stressors to make the weakness known.

Lettitia