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]

Your doctor's right in that 2.4 cm is the maximum for her size. 2.8 cm is way above that. She really should be evaluated by a highly qualified pediatric cardiologist. Which city/state do you live in?

[faxmom]

No, she has very normally spaced facial features and normal skin. I bruise easily, but she does not.

[nickppatel]

By the way, not all connective tissue disorders manifest with characteristic "Marfanoid" features. Does your daughter have widely spaced eyes and a split uvula? If so, she should be evaluated for Loeys Dietz syndrome. Does she have very loose, easy-bruising skin? She might be evaluated for Ehler-Danlos. It's very difficult to say without seeing her face/limbs.

[faxmom]

I think she's about 54" tall and 62 pounds...

[nickppatel]

What is her height & weight?

[faxmom]

Thank you so much for your comments. I think you're right. I should get a second opinion. There wasn't enough room to write everything in my post, but the Dr. did say that he often sees this with connective tissue disorders, like Marfan's, but it's "obvious she doesn't have Marfan's." I work with Medtronic (medical device company with lots of cardiac therapies, etc.), and I've actually met some patients with Marfan's. My daughter is petite--little feet, fine bones and has none of the facial features associated with Marfan's. I'm really not familiar with any of the other connective tissue disorders, but there are none--that I know of--in the family history. Do any of the others seem to fit the description? Also, I'm not familiar with how significant the dilation is. I saw that the report said the "high end of normal" was 24, so I'm not sure of the significance of 28? Does anyone else know? Thanks again. You are very kind to share your knowledge.