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]

Hi, no problem.

All of the signs you mentioned can be attributed to different connective tissue problems. Many of those signs occur regularly in the general population. But, given the fact that they rarely occur together in this way in the general population, I would say yes, seek the opinion of a pediatric cardiologist knowledgeable re: marfan and related connective tissue disorders. It's so important that you visit a cardiologist who knows a thing or two about Marfan and who will take your concerns seriously. Many doctors take a look at a patient and if the patient doesn't fit the classic image profile they see in textbooks, they assume the patient doesn't have the condition. Stay away from these types of doctors.

If you tell me where you live (City and state), I can provide you with name(s) of doctors unofficially endorsed by the National Marfan Foundation. There, you are likely to be seen by someone who knows what he/she is doing, and can order the appropriate tests for you kid to see whether Marfan is truly a possibility.

God bless,
Nick

[djgeno]

Sorry to comment this way but you seem very knowledgable.  I'll try to make this story brief.  My son has always had mild hypotonia affecting fine motor skills, maybe also affecting comprehension level and a 2 year delay from an educational perspective.  He is 12 yo.  Throughout his life we have had 2 CT of the head.  On both occasions the radiologist interpreted white matter migration.  Pediatric neuro shrugged off as poor quality films.  For the past 2 years I have quit searching for a diagnosis because he is developing and not regressing.  However, last week became short of breath, irregular heart beat, and chest pain.  Went to see MD. EKG and all electrolytes are normal but irregular rhythym could be heard.  I beleive it is called sinus dysrrhythmia which is common in children. Referred to pediatric cardiologist, but after putting all symptoms together I am concerned about marfans. He is positive for the wrist thumb and wrapping small finger and thumb around wrist with overlap,right and left chest uneven at the sternum, tall, lanky, high palate, crowded teeth, Has braces. And just recently within past year developed major nearsightedness.  We have been for eye checks numerous times with negative findings.  He is 64in tall and arm span is 67in. No family history to my knowledge. I called my MD to mentions these things but he has failed to return my call.  By the way he was stared on inderal 10mg twice daily.  Are there enough red flags to harass my MD?  I feel like a hypochondriac mother.  Thanks for input.    

[EchoTech]

Chest pain in general for children is completely different than adults with chest pain. The aorta is kind of the same game.  They are very different animals, hence you won't see adult cardiologists treaing children, and vice versa.  There are common exeptions but generally thats how it works.  There are specially trained cardiologists that see both children and adults, but not very many.

[nickppatel]

faxmom, I'm providing this link with the assumption that you are taking pbanders' and echotech's sound advice to let the doctor interpret what is and is not an appropriate course of action. Unfortunately, some doctors - like my old cardiologist - are poorly equipped to deal with these kinds of issues, and you should be equipped to spot them a mile away. So, take this with a grain of salt. It's a chart for aortic roots only, not for pulmonaries.

http://www.marfan.org/nmf/images/aortic_detection.gif

[kbheart]

If your daughter is still having chest pain, I would try to get her in soon for the second opinion.  I went to the ER with chest pain and they found a dilated aorta.  They sent me home and told me that they would check it again in a year.  It turns out that I was having chest pain because the dilated part of the aorta had already torn.  Their tests missed the tear.  My life was at risk for two weeks before they finally operated because they assumed my chest pain was just indigestion. Lots of people have connective tissue diseases like Marfan's without the outward appearrance of the disease, including me.  Take any chest pain very seriously and make sure your physicians do too.  Good luck! I wish you and your daughter the best.

[pbanders]

I wrote a long response, but I think short is better - ask yourself - should you be interpreting and reading aortic dimensional charts for your daughter, or would it be better if you had a pediatric cardiologist who you trust and can communicate with, reading the chart and properly interpreting the data in the specific case of your daughter's condition?

I don't think you need a chart - you need a different doctor. I went through the same thing and drove myself nuts before I found a doctor that I could trust and communicate with.