Daughter's "Dilated Sinus of Valsalva"

[faxmom]

Thank you so much for this wonderful forum. My daughter, who just turned 10, is extremely active--on a swim team and a runner. For the past 2+ months, she's complained that her "heart hurt" (an "ache and a burn" directly over her heart). She has also had a few incidents where she felt her heart was "racing" or "pounding out of her chest"(130 bpm max, however), and she has lightheadedness and pain at the same time. Not brought on with exertion or anxiety--both at school and home. Pediatrician did an exam and EKG and sent it to the Children's Heart Clinic with a referral. Met w/the cardiologist there last week, and he said the EKG/exam normal, and the racing heart not a problem, unless it goes up to 200s. Painpain was probably benign. To be safe, he ordered an echo.

Conclusions of echo:
1. The sinuses of Valsalva are diated for the patient's body surface area. Upper normal is 24 mm, and this patient's is 28 mm.
2. The main pulmonary artery appeared mildly dilated.

Dr. told us that he's not worried--just come every 1x every 2 years for echo. "No restrictions on activity, because if the aorta is going to erupt, it's going to erupt." I left rather shocked, and wished I had asked a million more questions. I'm wondering if this is a condition we should be worried about. Is there anything we can do to be sure it doesn't "erupt"? Should we limit activity? Is there anything else we should be doing? Should we get a second opinion? If this were your daughter, what would you do?Sorry for the long message, and THANK YOU in advance for your help. I really appreciate it.

[nickppatel]

pbanders -

my apologies. i re-read my post and saw language that was misleading ... the excessive activity in and of ITSELF probably wouldn't cause the dilatation; the activity combined WITH a predisposing factor WOULD.

Sorry,
Nick

[nickppatel]

pbanders -

Excessive exercise in those already predisposed to aortic dilatation, such as faxmom's daughter, can exacerbate the dilatation, due to hemodynamic stress on the weakened aortic wall. This is why most Marfan patients, EDS patients, LDS patients, or those with very large roots, etc. are placed on exercise restrictions as recommended by, for example, the National Marfan Foundation.

Kinoshita et al. published "Aortic Root Dilatation Among Young Competitive Athletes: Echocardiographic Screening of 1929 Athletes Between 15 and 34 Years of Age" a few years back. It is an interesting read and is available for free on medscape.

Nick

[pbanders]

Agree with nickppatel, you should get a pediatric cardiologist who can work with you on the identification of the cause of the dilation, which is likely due to a genetic cause (FAA, Marfan's, Ehler-Danlos, etc.). I had surgery to fix my ascending aortic aneurysm at age 46, I then had my two kids get tested and we've been monitoring them, I have no concerns about their near-term condition or activity level. BTW, I have never read anywhere (and I've seen a lot of references on this condition) that "excessive activity" can cause aortic dilation, if such references exist, I'd like to read them.

[nickppatel]

Get another cardiologist, PRONTO. Sorry, but that is hands-down the STUPIDEST thing I have EVER heard a cardiologist say.  "If it's going to erupt, it's going to erupt?" Are you KIDDING me?!

A doctor that paid attention in med school would take steps to figure out the underlying cause of your daughter's dilatation, eg, connective tissue disease. It is possible that the dilatation is due to excessive activity, too, or a combination of the two. A more sensible approach would be to limit your daughter's physical activity so that it isn't extreme and to monitor her with echos YEARLY. One might prescribe beta blockers to slow the dilatation. If your daughter has a connective tissue disease like Marfan, she might qualify to participate in the Johns Hopkins clinical trials with the drug losartan, which is expected to halt or possibly reverse dilatation. Or, once the dilatation is severe enough, surgery is warranted. But "if it's going to erupt, it's going to erupt" is just unbelievably bad advice.

Please, please find another cardiologist. You are getting awful advice. And if you want my honest opinion, if I were in your shoes, I'd consider reporting the cardiologist to a governing authority. I wonder if any kids have died due to this kind of advice. Can you tell I'm furious?

Anyway, there are a lot of knowledgeable people on this board who will add their 2 cents. If you want to contact me directly (since this thread will eventually be closed to further comments), let me know and I'd be happy to share what I do know.

Nick

[Forum-M.D.-bkj]

fax,

I'm not a pediatric cardiologist, so this question is somewhat out of my field.

I think we are all very concerned when it comes to illness in our children so I understand your concern.  I also understand how sometimes the wrong set of words can lead to undue worry in a parent or family member.  

I think you should seek a second opinion. I can tell by the tone of your post that this will weigh heavy on your mind and heart until it's sorted out.  If the second  opinion concurs, it will be much easier to focus on the health of your child.

good luck