Bicuspid Aortic Valve and Enlarged Aortic Root - 8 yr old

[TPittman]

My son will turn 8 this weekend and was diagnosed last year with a bicuspid valve and enlarged aortic root.  My husband has the same condition (he is now 39 and has experienced no problems).  Last year, our cardiologist told us that my son should be able to live his life without effect from this condition unless we saw a rapid increase in the size of his aortic root or other complications.  This year we saw the same cardiologist who reported that there was no change.  My son is 52 inches tall and weighs approximately 52 pounds.  His aortic root dilation is 27mm, the same as last year, which the doctor said was just over 2 standard deviations from the normal size.  He has no stenosis or regurgitation.  His blood pressure is good, and he has experienced no signs of any problems or issues.

My son is very active and currently plays baseball and wakeboards.  After our appointment, the cardiologist called us back a few days later and indicated that he reviewed the Bethesda Conference Guidelines (published in 2004) and believes that my son should follow the advice for competitive sports based on the Level IA of those guidelines, which restricts his activities to "billiards, bowling, cricket, curling, golf and riflery."   The higher levels that he should not particpate in include all other sports, including baseball, soccer, running, canoeing, skiing, and even table tennis.  We were really upset by this huge change of information, and are concerned about so severely limiting what our son can do.  We would like to try to understand what information we should consider when determining what activites our son can particpate in.  Can you give us some help understanding the risks involved?
Thank you so much for having this forum!

[Jeffrey R Boris, M.D.]

Dear TPittman,

The concept of aortic root dilation in the face of a bicuspid aortic valve is a relatively recently recognized one.  We do know that it is a complication of bicuspid aortic valve, as is the development of aortic valve stenosis, regurgitation, and prolapse.  At this point, though, we do not have a lot of data on the associated risk of exercise, especially in those patients whose aortic roots are just a bit bigger than 2 standard deviations (also referred to as a Z score greater than +2) than normal.  I think that there will continue to be increasing reports of aortic root dilation leading to aortic dissection and rupture, with need for emergent surgery or even sudden death.  However, we think that this is in association with severely dilated aortas.  Certainly, if you are saying that the aortic root measurement is unchanged from last year and he continues to be observed, it is hard to justify severe exercise limitations.  To be sure, bicuspid aortic valve used to be a historical curiosity, with plenty of patients living to much older age without any complications from it, and having it found incidentally during autopsy while having died of something else.  Overall, I think that a lot of cardiologists would be comfortable saying that competitive isometric activities (high school level football, wrestling, weightlifting, etc.) would probably not be a good thing to do, but that the rest of physical activity would be fine, especially at his age.  At the age of 8 years, he isn’t even able to do what would be considered “competitive” isometric activities.  I do think that it is probably not a good idea to let kids do things like, “play football now, then stop later;”  I think that is confusing to them and unnecessarily gets their hopes up.  There are plenty of other great athletic opportunities that would not require eventual discontinuation.  Finally, understand that the rapid progression of the aortic root dilation may end up being grounds for subsequently limiting participation.  That said, I did discuss this with the head of our exercise physiology laboratory, who brought out two very good points.  One, the Bethesda Conference specifically discusses competitive athletic participation in patients 14 years of age and older; neither of these criteria apply to your son. Two, the adult literature says that an adult with a bicuspid aortic valve and no stenosis or insufficiency and an aortic root of 4 cm or less needs no restrictions.  Therefore, I would talk with your cardiologist and ask on what basis of proof his recommendation has been made.   It is certainly not unreasonable, though, to obtain a second opinion for this if you feel that this recommendation is not sound.