Diagnosing a BAV

[FredW]

This post is directed to those who have been diagnosed with a bicuspid aortic valve. My research on BAV tells me one can be diagnosed early in life or late in life, after stenosis sets in. I also understand some can live their whole life and never know it. It appears it is confirmed or picked up with an echo test or a TEE test. But it does appear that there is a possibility that echo tests do not accurately detect a bicuspid valve.

I have had three four echo tests in the past two years to monitor my AS. The third test showed that I may have a BAV and the cardiologist ordered a TEE. That test showed I did not have a bicuspid valve. Inconsistencies in my last echo and catheterization caused me to seek a second opinion with another cardiologist. He gave me a stress echo test and suspects I do have a BAV and ordered a CT scan. Hopefully the results of that test will confirm my condition, for it is frustration not knowing exactly what I am dealing with.

My question to those of you that are dealing with a BAV is how was it diagnosed and has anybody had similar experiences that I am having getting an accurate handle on their valve issue.

Maybe it is the the math/engineer in me that wants accurate answers when I should not expect them. Am I expecting too much?

Guess I should just get comfortable in the waiting room, for I may be here a while. Meanwhile, I get very frustrated when I can't work for over an hour cutting up a fallen tree with my chain saw. That caused dizziness and exhaustion that drove me to the couch and a hour nap.

Always asking questions,
Fred

 

[Paleowoman]

Hi Fred - I was diagnosed with bicuspid aortic valve in my late 20's. The murmur was actualy heard when I was 25 but echo then did not show it. Another cardiologist, another echo, showed it about four years later. But nothing was done because it was not stenotic, not causing any trouble at all, it just made a murmur whenever a doctor listened to my chest. I never saw another cardiologist until my early 50's by which time the valve was moderatley stenotic. Had surgery when I was 60 when the valve became critically stenotic. I never had any symptoms though, not even just before surgery (did weight lifting and a five mile walk day before surgery, no problem). Apart from taking nearly a year to recover from surgery, I feel the same now as I always felt - strange in view of others' experiences, but might be because I'm in the UK and they tend to do surgery when the stenosis is critical but before any symptoms develop.

 

[Warrick]

Hi there, I'm 39 years old now, well for me I broke my hand about 8 years ago and when I was in hospital having the bone aligned the nurse asked me do I know I have a heart murmur? I was in the army and had left 3 years or so earlier and never had a murmur, I had all the usual annual medicals and nothing was ever picked up. So I had a thoracic echo a few months later, I rung the gp as I hadn't heard news and was told over the phone everything was fine.
4 years later I was at the gp (I hadn't been to the doctor in those 4 years) for a medical for my heavy truck licence and I asked the gp about the echo, he explained the way the valve was mis-formed but never mentioned the term "bicuspid aortic valve", he told me it was nothing to worry about and have it checked again in 10 years time, he got that wrong!!!
A few months later I googled it for the first time and found the term bicuspid and a lot of info on the Cleveland clinic site, and of the possible need for surgery. Probably 2 years after that I noticed I was struggling more than normal on hill climbs when hunting especially one particular walking track that I hadn't done for a year but that made me realise things weren't right, I held off going to the doctor for a long time but then in June last year bit the bullet and went to the doctor.
Saw an American locum instead of my usual gp, he listened and said "there's a significant amount of leakage there you're going to have that replaced" so had a thoracic echo, saw the cardiologist in august last year, I was told they couldn't see well enough so needed a transesophageal echo and I may or may not need surgery. Had the transesophageal echo on the 22nd September and it was confirmed that it was stuffed and leaking badly. Apart from the hill climbs being hard work and a bit of shortness of breath every now and then I felt ok. Had the surgery workup and went under 4th November 2015 and had a 27mm St Jude mechanical fitted. I haven't really had a doctor tell me anything of the technical stuff like amounts of leakage etc. before surgery or of my prognosis for the future as I still have an aneurysm (4.16 cm) but this smaller than the surgery guidelines it seems even though they were in there, they seem reluctant to discuss anything negative, tell me sqat and here in NZ you can't just make an appointment with the cardiologist (I guess everywhere else is the same?)you wait to be summoned, but I've got copies of the reports and found what I do know online, and from the fantastic people here!!

 

[epstns]

Hi, Fred, and welcome to The Waiting Room - the virtual room where many folks await their own turns at valve surgery. I spent nearly 10 years in The Waiting Room myself, so I can imagine how you feel.

Keep in mind that the diagnosis of a bicuspid aortic valve, in itself, does not tell you if or when you may need valve surgery. I'm writing from memory right now, but IIRC, about 2% of the population of the world have BAV, but only about 25% of those (the 2%) will have symptoms significant enough to warrant surgery. So, those of us who do have surgery must amount to about 0.5% of the world population -- we are rare gems!

Bicuspid valves primarily go "bad" in one of two ways. Either they become stenotic (aortic stenosis) and do not open fully, thus keeping the heart from supplying enough blood to the body, or they become regurgitant, not closing fully, and allowing blood to flow back into the lungs rather than flowing out to the body. An echo can tell you if you have either of these conditions usually before they become severe enough to cause symptoms.

I had a "probable" bicuspid valve. It was never visualized clearly enough on an echo to know for sure, but it really didn't matter. I was originally diagnosed with moderate to severe aortic stenosis at around age 52. We monitored my valve and I went on with life - running, going to the gym 5 days a week, working a stressful professional job, traveling, raising a family. . . just a normal life. Until my valve got bad enough that I felt it was time to replace it. My cardio had told me that for the 2 or 3 years prior to surgery my statistics were bad enough to warrant surgery but that I was doing so well that he could not, in clear conscience, recommend surgery. He said "You will tell me when you are ready." I did -- I was "tired of being tired."

I had my valve replaced nearly 5 years ago, at age 63. I won't bore you with the whole story (although if you want to know, just PM me), but after recovery I went back to doing everything I used to do, only better and harder. I'm still at the gym 5 days a week, although I can't run any more (knees). I am so grateful to have found the "family" I have here, as they are the most knowledgeable community with regard to heart valve issues, and they are very open and happy to share their experiences.

Again, welcome. Stick around a bit and ask all the questions you want. You've come to a wonderful place to get the answers you need.

 

[Superman]

Fred,

Allow me to echo everyone's sentiments in welcoming you aboard.

When considering BAV, it is important to know that it isn't a black and white condition. It is not like a normal valve has three perfectly proportioned equal leaflets, and a BAV has two perfectly proportioned semi-circular leaflets. There are varying presentations of a what a "BAV" will look like. Some will be barely detectable in an echo (not enough resolution). Some may actually only have one leaflet, but be included in the condition we know as BAV.

One of my children, for example, has three leaflets, two of which are partially fused. He has no symptoms, no murmur, no regurgitation, and no stenosis. In effect, the valve is what I call "functionally normal". While technically being diagnosed with BAV, he may never face any intervention or limitations. We might have never found out about his if it were not for me. My valve, on the other hand, was a mess from the day I was born. I honestly don't know the exact proportion of the leaflets, but I do know that I had both stenosis and regurgitation from a very early age. As far back as I recall, I was told that my valve didn't open all the way, and it didn't close all the way - so I couldn't participate in sports or gym. I was well enough to wait until my late teens to have my valve replaced, but there was talk of it as early as 12.

Hope that helps.

 

[FredW]

What great feedback. Thanks all for sharing their story. I'm still soaking in the information and learning a lot from this forum.

I got a call from my second opinion cardiologist (who I'll refer to in the future as my Cardiologist). He read the TEE test that was done last July and said that it looks like I do have a bicuspid valve - that is is subtle , but two of the leaflets are fused. The recent CT scan report said I have three leaflets and no aneurysm, but the doc, based on the TEE test, is fairly sure it is a bicuspid valve. Apparently, this has not been problem for me all my life until I developed stenosis which showed up with a mummer about two years ago. The course of action is the same: continue to monitor it until the stenosis gets serious enough to warrant surgery. As a side note, when I told the doc about getting dizzy from using the chain saw, his reply was: "Why are you cutting up a tree when you have a heart problem? If you had a bad back, you wouldn't go out and lift 100 pounds." So, guess I have to change my life style some until I get this fixed.

Steve suggested I stick around and ask all the questions I want, so I'll pose this question: BAV is a [FONT=arial, verdana, lucida sans unicode, lucida grande, helvetica, sans-serif]congenital condition and it is suggested that one's family get monitored for it. I have four children that are genetically connected to me and a number of nieces and nephews. There are few stories of 'heart' issues in my parents family and my siblings died early due to bad life choices (cancer from smoking, liver failure due to drinking, Heptitus C from drug use). So I'm the first that has been told to have a BAV. I figure it is my responsibility to warn my serving family to be on the watch for BAV. Any suggestions on how to do that? [/FONT]

 

[Superman]

Just tell them. "I have been diagnose with this condition. It is a hereditary condition. It would be a good thing for you to be tested." What they do with it is their business. My family would not get tested (and still hasn't been to my knowledge) even though we set up participating in a study that would have covered the cost of their testing. I did have my kids tested. I can only control what I can control.

There really wasn't much talk of the genetics of it when I grew up. I was born with a defect, and nobody else had it. I never considered it to be hereditary since nobody in my family had heart surgery or even saw a cardiologist outside of my grandfather who passed at 80 from congestive heart failure. But that seemed like a long, healthy life for the most part. He was one of ten, and they all lived to around 80 or longer.

 

[Seaton]

Greetings dear Fred. And welcome.

I, too, am in the Waiting Room.

I stumbled upon my BAV last February after being sent for an echo due to continual palpitations and increasing fatigue in the preceding months.

I’ve had a murmur since childhood and though lots of doctors had mentioned it down the years it seems none thought to connect the dots and asked as to its probable cause.

I’d always assumed it was just a quirky heart difference, something I could walk safely through life with. But alas …

The echo last Feb detected a ‘bicuspid aortic valve’. I’d never heard of it. I went home and Googled it and suddenly the future had a slightly altered narrative to the one I had vaguely imagined for myself (emphasis being on the vaguely). But viva the fact they found it when they did – and gratitude for the wonders of modern science.

They also diagnosed a mild aortic root aneurysm – such aneurysms often being a particular accompaniment to BAV construction.

My consultant(s) in London have written in my notes that my valve is ‘functionally bicuspid’ with moderate-severe aortic stenosis. I’m currently being monitored after a bank of tests last year. The tests will be ongoing (I have another treadmill cardiac test and possibly an echo next Thursday).

It’s watch and wait for me, it seems.

My ‘indexed estimated valve size’ (relative to my height of six foot) is 0.5 cm² according to my last appointment in October. This figure seems critical to me, but it seems my specialists think because my ‘peak/mean gradients’ are still relatively low, and I have displayed no major symptoms, they prefer to continue to monitor. So trust it be. They're the experts. I’m in their very capable hands.

I have two beautiful grown-up daughters. They were more than happy to have themselves tested for BAVs once I explained the reasoning behind it. They’re pragmatic people. The good news is that both were tricuspid. So all’s well.

I wish you the best as you more forward with this. And am around here writing and reading posts. And at your service in any small (or large!) way I can. ;-)

A cheer from London.

 

[Zoltania]

For what it's worth, ever since I started reading this forum and seeing the abbreviation BAV, I have been involuntarily mentally interpreting it as "Bad-Ass Valve."

 

[MrsBray]

I was diagnosed with aortic stenosis. The BAV didn't even come in to the conversation for 5 more years. I think it's really tough to say for sure until the surgeon actually sees the valve. I "got" my murmur at age 33, during a DOT mandated physical. Physicians before and after missed it. My first cardio didn't even mention BAV. I was frustrated initially because there were no concrete answers. You're definitely in good company here.

 

[cpj123]

Fred,

I was 52 before they ever heard a murmur...then about 3 echos...TEE... Then a CAT said tri-cuspid. In the end the regurgitation was such that I had to go ahead and do it. The end of the story is that it was BAV but it was never confirmed until surgery.