Insight? BAV w/ aortic aneurysm

[jbrett]

Hi all!
First, I'll give you a little background info: I'm and 33 y.o. mom of two and I've known of my BAV since I was about 12 or so. I've never had any restrictions on activity or anything and I've had two easy pregnancies. My BAV was always in the back of my mind, just as something that I'd probably have to have replaced when I was much older. I've lived a normal life and have somewhat kept up with my cardio visits, which were always routine. However, after not going to the cardio since, oh, 2008 (I'd had a baby in 09- guess I've had other things on my mind!), I made an appointment and finally went back in Feb or March of this year. My cardio at that time had mentioned that since it had been a while since I had last had an echo, he wanted me to do that asap and also schedule an MRA to have a better look at my aorta. Not because I'd been having problems, but because he wanted a baseline measurement. So I did all of that and then a few weeks ago, not expecting anything wrong, I went back to see him to discuss the results.

Turns out that I have a 4cm dilation/aneurysm on my ascending aorta, which, since I'm only 5'1" and fairly small is pretty big, I guess. He now wants me to have a scan every 6 months (echo alternating with MRA) to watch and see how it's growing. Unfortunately at my last echo in 2008 they didn't measure the ascending aorta. He also started me on a low dose of a beta blocker (metoprolol). Fortunately, however, my valve function is good- only very mild thickening and stenosis.

Since that appointment, which was a lot to absorb, I've had plenty of time to think, research and read. Probably too much! (no, definitely too much). One of the things that my doc said was that he was going to consult with some other docs about my aorta size in relation to my height/body size. So I've researched that too, online and through a friend who's a nurse practitioner. And what I've come up with is that my value for the Aortic Size Index is 2.5 (there's a cool calculator here:http://valleyheartandvascular.com/Thoracic-Aneurysm-Program/Calculate-Your-Relative-Aortic-Size.aspx) and I've found that one of the indications for surgery to repair the aneurysm for a BAV patient is to do it before the ASI reaches 2.75, which, for me, is 4.4cm(!).

Is this something that any of you have heard of? I'm not eager to go under the knife at all, but feeling like I've got a ticking time bomb in my chest isn't pleasant either, as I'm sure many of you can relate! Also, would they do surgery if my valve is still operating well? Also, symptoms seem to be a tricky thing, especially since BAV is something that you're born with it's difficult to know what's normal or not. I can say that while I've always been active, I've never had great endurance. Sometimes I think that I'm experiencing shortness of breath, but I've always attributed it to stress or not working out enough. I've also in the last six months started to get migraine auras (no headache), but my cardio doesn't think that this is related (I'm not so sure). I have a follow up appt. in 2 weeks to see how my beta blocker is doing. I also plan on asking him lots of questions!

Anyways, sorry for the long post! Reading through your stories has been really helpful for me- best wishes to all of you! Any insight any of you have is most appreciated!

 

[camgough]

Hi there. I'm 31 year old male and had surgery July 2010. Just before surgery I found out I had a BAV with a 6.7 cm aneurysm. My valve was working well too and I had valve sparing aortic root replacement surgery. They fix the aneurysm and left my valve in there. Seems silly to do that since I will likely need another future valve replacement surgery, but this way no need for blood thinners. This may be something you could consider. Good luck and let me know if you have any questions.

 

[Bill B]

jbrett:

Yes, these aneurysm to body size indexes have been mentioned here before. So, right now you are below the indication for surgery. Although these things do tend to progress, there is no way to predict the rate of progression. You might be perfectly stable for quite a while or not. So, wait for the next test in 6 months. Whether your symptoms, including the migraines, are related to your BAV is something your doctors will have to tell you, among you other questions. It sounds like your valve is not too bad. Also, the aneurysm itself does not cause shortness of breath. Enlarged segments of the aorta are common in BAV - just part of the connective tissue disorder. My valve was in horrible condition and required surgery. Although my aorta may not have met the indication for surgery at that time and might have been stable for a lot longer, I opted to have the repair done to avoid the high likelihood of another surgery at a later time.

 

[skeptic49]

Thanks for posting the link to the calculator. Even for me, a relative giant at 6'5", with BAV the trigger point is at 5.0cm. Very interesting!

 

[ElectLive]

The cross sectional area formulas were developed precisely for people of short stature after clinical review of cases of aortic dissection showed it happening earlier in shorter individuals. The Cleveland Clinic (#1 rated adult heart center in the US) uses the formula on the right hand side of that link you posted, with 10 being the threshold for surgery. The head of the Aorta Center at the Cleveland Clinic, Lars Svensson, has written a few clinical journal articles/studies discussing the issue. The national practice guidelines (Thoracic Aorta Disease) for diagnosis and management of patients with aorta disease (which includes the connective tissue disorder and aneurysm component of BAV) also specifies the cross sectional area formula as a means for determining surgical thresholds.

It sounds like in your case the aorta may determine when operation is needed, in many cases it does, as was true for Cam and also me. My valve situation was slightly frustrating since it theoretically could have waited a few years, but certainly not long enough to make a valve sparing operation a reasonable solution, so at the end of the day, it was a pretty easy decision to do the valve at the same time. No one ever wants to replace a valve prematurely but when the alternative is to postpone a necessary surgery on the aorta, waiting on a valve is doing nothing but adding risk.

I was diagnosed at birth and had just sort of been floating along with boring echo after boring echo, always being told something might need to be done (valve replacement) eventually, later not sooner. Then, I had a 5.0 cm aorta measurement come out of nowhere (rapid growth) so, yes, I know the ticking time bomb feeling well. But many also remain stable for years, and you are much better off to be aware of the situation early. Beta blockers and frequent echo/CT/MR monitoring are standard procedure, so you are already well taken care of.

Even if things progress, you still may not have any symptoms, surprisingly, it all depends on the individual, and I had none. Best wishes to you moving forward. You have found a great place to ask questions, and hopefully for as long as possible, you will just be here to wait and learn.

 

[jbrett]

Thank you all so much! It's been a roller coaster few weeks, but I have learned a ton of info and really appreciate everyone's thoughts on the topic. My BAV went from something that I rarely even thought about to something that's been totally preoccupying me. Well, the aneurysm more than the BAV. I like to go into any medical situation with my eyes wide open, so even though I'm hopefully a ways away from needing surgery, at least I understand what's going on, what could happen, and who the specialists are in my area.

One more question though- Are most cardios familiar with and accepting of the ASI calculation?

Thanks again!

 

[Bill B]

One more question though- Are most cardios familiar with and accepting of the ASI calculation?

Thanks again!

Sure, I would say most are. More so, the surgeons would be. But remember it is a statistical estimation based on historic information. My bias is that I'd rather be early than late fixing something like this. Of course, not so early that it is completely unnecessary, but as it approaches, especially if the changes are rapid, my bias would be jump on it. The calculation doesn't include rate of change, and like most of medicine, treatment is a judgment call, a combination of things, and shouldn't rely solely on a specific number. And the condition of your valve is another variable in timing your OHS and figuring out what the best approach would be.

Hang in there. Try to enjoy life in the meantime. These things are very fixable and the surgery is remarkably routine and very safe. So, keep doing those periodic echos until, when and if, it is clear it's time to get it fixed. Then be confident that your surgeon will take care of this.

 

[Richard Gardner]

I didn't know anything was wrong with my heart until three years ago at age 57. A visit to the doctor for something unrelated uncovered a heart murmer. An echocardiagram showed a leaking bicusbid valve and "mild enlargement" of the aorta. I was referred to a cardiologist who wanted to take a better look. As I was training for an Ironman Triathlon I couldn't come up with a time when I would be free for the testing. He agreed to wait until after the race, 3 or 4 months later, to do the testing. A T.E.E. and MRI showed my aorta had reached 5.3 centimeters. I guess that's pretty big for me since I too am rather short (5ft. 4in.)) Since I had just started a new job I needed to wait 5 months before my health insurance kicked in. I had my aortic valve replaced (porcine) and the upper aorta replaced at that time. I never had any symptoms throughout all of this and the surgeon said he had never seen someone present as well as I did with as much calcification of the valve leaflets.
I also had migraine auras without the headaches for years and still get them now.

 

[jbrett]

Hi Richard- thanks for your story, and congrats on the Ironman. My brother completed one a few years ago, so I know what's involved. It's a huge accomplishment. Although as far as I know, my bro doesn't have BAV, I am urging him to be checked for an aneurysm. Just in case.

I find this migraine aura thing so interesting. I'd never heard of them until I started experiencing them about 6 months ago, but it seems from looking around on the boards that they are somewhat common.

 

[ElectLive]

Although as far as I know, my bro doesn't have BAV, I am urging him to be checked for an aneurysm. Just in case.

Good for you, that's very important. It sounds like you already know this, but just in case, any first degree relatives are potentially at risk of aneurysm development even if they don't have BAV. And the fact that first degree relatives don't have a murmur doesn't necessarily mean they don't have BAV either, it can be missed, then show up as a "surprise" after an echo is done.

 

[mom of 5]

Thank you for the link ! I

 

[Jason]

Count me as one that had an enlargement of the aorta present and then stabilize itself for quite some time. In 2001 or 2002 (I don't remember which) I had an annual echo due to my BAV from birth. At that time my ascending aorta had jumped up to 4.0 or 4.2 so I was put on a watch. It stayed there for the next ten years, until last year it was at 5.4 cm. I had the surgery, and since my valve was deteriorating anyway they replaced it with a mechanical. The aneurysms can stabilize, but eventually mine got to a point where I needed surgery. Keep reading and learning!

 

[jbrett]

Count me as one that had an enlargement of the aorta present and then stabilize itself for quite some time. In 2001 or 2002 (I don't remember which) I had an annual echo due to my BAV from birth. At that time my ascending aorta had jumped up to 4.0 or 4.2 so I was put on a watch. It stayed there for the next ten years, until last year it was at 5.4 cm. I had the surgery, and since my valve was deteriorating anyway they replaced it with a mechanical. The aneurysms can stabilize, but eventually mine got to a point where I needed surgery. Keep reading and learning!

Hi Jason, thanks for your input! It's good to know that AAAs can stabilize for so long. I'm starting to finally understand why cardios seem to want to hold off on fixing them for as long as they can. Especially if the BAV is functioning well, like mine. During the time that you were watching and waiting, did you have any restrictions placed on you? Did you just live life like normal? Did you travel and did you have any problems getting/keeping health insurance? How often were you scanned?

So sorry for all of the questions, but I think that these are my biggest concerns with the whole waiting game. AAAs don't fix themselves, so I'm pretty sure that I'll eventually need to have surgery. But as you can probably tell, I'm terrible at waiting, hate surprises, and am really not used to having something "wrong" with me If nothing else, all of this will probably teach me some patience and inspire me to exercise more regularly!

jen