Hi.....New here! Son with BAV

[B's Mom]

Hi all!

I have been lurking since 2010 when we found my 7 year old son had BAV and mildly dialated ascending aorta. This is such a great place to be if you need support or have questions. First, we found my sons BAV "by accident" when he was being screened for problems from strep....which were negative. He had no murmur or any other symptoms. We would still have no idea if it weren't for this screening. So, for that, I am grateful. Anyway, in 2010, first echo showed BAV, no stenosis and trivial/trace insufficiency and mildly dialated ascending aorta at 2.6 cm. He just got his 2 year check-up last Thursday and there is still no stenosis, trivial/trace insufficiency, however aorta showed more enlargement. Here is where I get confused and I am hoping the veterans on here can shed some light. Well, first the doc came in after peeking quickly at his echo and called the aorta moderately dialated now. He restricted very heavy weightlifting and nothing else. I wanted some numbers but he didn't have them at that point. A few days later I obtained the test results.......echo measurments and the doc summary to primary physician. The echo results from tech say.....no stenosis, no evidence of aortic insufficiency, and moderatley dialated ascending aorta of 3.1 cm. (up from 2.6 two years earlier) BUT, the summary the fellow wrote about the echo (actual doc did not view...just his fellow)..stated no stenosis, trivial insufficiency and mildly dilated ascending aorta at 2.8 cm. Why the two different readings?? Which is more accurate?? And..... What are "extreme valsava maneuvers"? (from which my son is restricted.)

Thanl you so much!!

 

[Fundy]

definition from wikipedia http://en.wikipedia.org/wiki/Valsalva_maneuver

The Valsalva maneuver or Valsalva manoeuvre is performed by moderately forceful attempted exhalation against a closed airway, usually done by closing one's mouth and pinching one's nose shut. Variations of the maneuver can be used either in medical examination as a test of cardiac function and autonomic nervous control of the heart, or to "clear" the ears and sinuses (that is, to equalize pressure between them) when ambient pressure changes, as in diving, hyperbaric oxygen therapy, or aviation.

 

[Superman]

Given the heavy weight lifting restriction and the definition provided by Fundy above, I have a guess. Probably means restricted from lifting so heavy he feels like he has to hold his breath through the lift. So maybe not an actual pound restriction, but if he can't breath all the way through lifting something, he shouldn't lift it?

Regarding the different readings on the aorta, there is a pretty significant margin of error on an echo. The readings can vary with the technician and the doctors interpretation. I had an echo that showed 4.3 cm and followed it with a CT scan (more accurate) that showed 4.9 cm. That's not to say your sons is worse than it shows. It just means that when it comes time for intervention, they will order a more accurate test before they do anything. Right now your son is just in monitoring stage. That's good. Many people make it will into adulthood (40's or 50's plus) before needing surgical intervention and many more don't need it at all.

Don't look at my info below and get scared. I had a severe murmur and was stenotic from infancy. One thing I've learned on this site that has given me comfort with my kids is that my case seems to be rather exceptional rather than the rule.

 

[Lynlw]

Hi, Sorry for your reason having to join, but glad you found us. As the others said, echo measurements aren't really exact and depending where and even a slight difference in angle when they are measuring can give slightly different results and different techs or different docs can come up with slightly different measurements on the exact same echo.

Regarding the restrictions, like superman said, the valsalva that they are talking about for restrictions, has to do with with lifting heavier things, Its tough giving weights as numbers like dont lift over 50 pounds or 20 pounds with children who are growing and what is "heavy' isn't the same each year, or even the same for all kids of a certain age, since they come in so many different sizes , usually at least in our case and others kids I know the doctors usually say don't lift anything you have to bear down or "grunt' to lift-. THAT is too heavy and as long as you stick by that growing up and even as an adult, they won't lift anything that is too heavy wether that is 20 pounds for a small child or maybe 100 pounds for a large adult male.

Kind of like what is too heavy to lift, measurements of the different parts of the heart and when you need intervention also isn't as cut and dry in children who are growing as it is in adults. like 5.0 is a measurement most doctors reccomend surgery in adults, they have to take everything into consderation when a small child is growing, and how it relates to all the other parts, I'm not explaining this well, but when watching 1 part like the ascendning aorta, since it will get larger as the children grow and everything gets larger, how does that compare to the rest of the Aorta or rest of the heart. kind of like was the ascending Aorta only slightly larger than the rest of the Aorta 1 year, but now is twice as large as the other parts now type of thing. They have all kinds of formula's and charts like how does that compare to other children the same size.
Now for stenosis its a little easier, in that you can keep an eye on the different pressures and gradients,( pretty much the difference in pressure measurements from one side of valve to the other) what pressure is "too high" is the same in a tiny person and heart as it is in a large adult

 

[ElectLive]

Well, everyone has done a great job already on explaining Valsalva, but just wanted to reference one medical source for this instruction - the ACC/AHA Thoracic Aorta Disease Guidelines:

"Patients often ask exactly how much weight is permissible to lift. Unfortunately, it is not possible to provide a blanket answer to that question, as it all depends on the patient’s size, muscular strength, physical
fitness, and how the weight is actually lifted. Rather than try to define a numerical limit, it may be useful to explain that patients can lift whatever weight they can comfortably lift without having to “bear down” or perform the Valsalva maneuver."

 

[B's Mom]

Thank you everyone! So, as far as restrictions. I specifically asked about pull-ups, sit-ups, push-ups and running long distances(heart pumping hard) and the docs said it was fine. Wouldn't that stuff be bad? These things are not done regularly, just will begin next year for physical fitness testing so the pull-ups etc. will be done 2 to 3 times per year. Just not sure. They are assuring me there is nothing to worry about righ tnow.....easier said than done when you are the parent! Ugh! Oh...and baseball. My son is a big time baseball player and plays 6 months out of the year....pitcher. They also said that wasfine.

 

[Fundy]

One thing you might want to consider asking your doctors about, is the benefits of BAV persons with anneurysm taking losartan to strengthen the aorta. There have been a few mentions at this site concerning a theory and studies of Hal Dietz regarding persons with Marfan Syndrome and the benefits of losartan. He also suggests it should help persons with BAV as well.

One mention of it at this site is here:
http://www.valvereplacement.org/for...rtan-Cozaar-for-BAV-with-aneurysm-says-expert

If I remember correctly I think the member starting that thread(Maryka) believes it inhibited her anneurysm from enlarging after taking losartan. I also think I remember others saying their anneurysms continued to enlarge while taking losartan. I have no opinion myself whether it helps or not.

However, you still may want to ask about the Dietz recommendation that BAV persons with anneurysm should take losartan.

 

[Joe]

Welcome - Sorry to hear about your Son. But I'm sure you will find support here - hopefully along with some answers.

What I've been told by my cardiologist about weight lifting is that it causes increased blood pressure throughout your body when you go to pick up that heavy weight. It's not just your muscles in your arms and legs lifting its also the muscles that surround your vessels that constrict. Long story short when your pressure spikes like that - it adds additional stress on your valve.

The changes in the echo happen, yesterday I got my first echo reports - and they were much more grim then my current. So don't be too shocked to see them change & possibly get "better."

Have you got more then one opinion about your sons condition? There is a lot of comfort when you have two highly trained individuals who don't know each other agree on the diagnosis / prognosis.

 

[ElectLive]

Thank you everyone! So, as far as restrictions. I specifically asked about pull-ups, sit-ups, push-ups and running long distances(heart pumping hard) and the docs said it was fine. Wouldn't that stuff be bad? These things are not done regularly, just will begin next year for physical fitness testing so the pull-ups etc. will be done 2 to 3 times per year. Just not sure. They are assuring me there is nothing to worry about righ tnow.....easier said than done when you are the parent! Ugh! Oh...and baseball. My son is a big time baseball player and plays 6 months out of the year....pitcher. They also said that wasfine.

These questions you bring up are somewhat of a dilemma for our medical professionals as well, and a reason why many of us receive completely different advice even for nearly identical conditions. For those with aneurysms or previously repaired dissections, there is much more agreeement: moderate aerobic exercise encouraged, vigorous aerobic exercise with caution and monitoring, and generally no heavy isometric exercise (lifting, etc) allowed. For everyone else, though, such as your son, that just have a BAV and at worst mild worsening of either the valve or aorta, I'm not sure there's really any consensus on where the line should be drawn between prevention versus encouragement. This is in many ways true for those of us who have had a BAV replaced and/or aneurysm repaired also.

For what it's worth, when I was your son's age, about 30 years ago, I was told to not do pull-ups, push-ups, chin-ups, or any strenuous weightlifting. I had BAV, but no stenosis or aorta dilation at the time. I was young enough then to just do what my parents said (following pediatric cardiologist orders of course), and never really thought much about it, other than of course when I was the only one not doing those things in gym class. I did do competitive basketball and everything else, though. But anyway, do I think my doctor was being overprotective? Perhaps. Is there really a need for these restrictions prior to true valve or aorta dysfunction? Perhaps not. My aorta and valve went bad still pretty early in life. I guess there's a chance it might have happened earlier if I'd done any of those things I was told not to, but I'm not really 100% convinced, and I don't think any cardiologist would be either...they just don't know for sure. But I do think the most important issue for any of the exercise restrictions usually has more to do with worsening of aneurysms already there, and that's why there are pretty direct guidelines for those patients.

As a a parent, I completely understand your perspective. I'm fortunate that neither of my girls has BAV, but they are still at risk for an aneurysm, although fortunately no evidence thus far, but still also very young. If I'm faced with any of these same questions later on, I'm sure I'll be overprotective to a degree but will probably just do my best to try and find the right balance, emphasize the moderate so kids can be kids without taking on unnecessary risk.

If you want to read a little more about the various types of exercise and the medical reasoning behind many of the restrictions, please see page 81 (e344) of the guidelines I mentioned previously: http://circ.ahajournals.org/content/121/13/e266.full.pdf+html. Again, this is not directly applicable to your son's current condition, and I hesitate to post it for that reason, but it is the best reference point I've found to explain more fully the impact of all types of exercise on the aorta, so thought it might be useful to you at least for that. Best wishes to you and your son and hope to hear he's pitching in the Major League one day.

 

[sarahsunshine]

I thought I’d pipe in.

My step-son (12yo) just had his mitral valve replaced for BAV, regurgitation and stenosis in November 2011. His valve actually was only “mild”. On the other hand, he had a mechanical mitral valve that did need replacing, and they figured they’d either repair or replace the Aortic Valve while they were in there to reduce future surgeries. While they were in there they discovered that his ascending aorta was >5cm. They replaced it with a graft.

You seem to be in a similar situation (without the mechanical mitral valve), except that you know about the ascending aorta pre-surgery. We had no idea. When we asked Skyler’s cardiologist on Tuesday why we hadn’t known pre-surgery, he said, “Really? That must have been a communication mistake on our part. I’m sure we knew.” Who knows if he is covering it up or not.

As most people will tell you, what is more important is the rate of increase of the aneurism, rather than the actual size. I do know that Skyler was having a ton of symptoms pre-surgery (many related to an undersized mitral valve), but I think that chest pain and extreme shortness of breath from doing very minimal activity (walk up 8 steps) was largely due to the aneurism (personal opinion). He couldn't get his heart rate elevated to where they could test him for the modified stress test! Does your son have any symptoms such as chest pain, shortness of breath, dizziness, related to intense exercise? If so, then moderation is key, and talking to his cardiologist.

If he has no symptoms, I would worry less, but keep up on the visits to the cardiologist (how often do you go? Every 6 months?), and ask specifically about your concerns about the growth of the ascending aorta. Get the numbers from past Echos, track how it has changed over time, and bring them up with your doctor. It’s possible that it has been overlooked. I’ve found that good doctors LIKE IT when you know about your condition and have specific questions to ask. DO IT! And if your doctor blows you off – get a second opinion and someone who will take the time to answer you concerns and takes you seriously.

Lastly, I understand that it’s tough to have a child who has heart issues. Don’t focus on the things he “can’t” do, but on the things he can! It sounds like you have a terrific kid. Enjoy him!