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Daughter's "Dilated Sinus of Valsalva"
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.
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.
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
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
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
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
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.
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
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 -
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
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
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.
What is her height & weight?
I think she's about 54" tall and 62 pounds...
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.
No, she has very normally spaced facial features and normal skin. I bruise easily, but she does not.
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?
I'm in the Minneapolis area, so I know that I can go to Mayo Clinic in Rochester, if necessary, or even Cleveland Clinic, if that's what I need to do. However, the cardiologist we saw made it sound like such a "no big deal" thing that I thought I must be just a overly protective/worried mom. Of course, as you can tell, I wasn't willing to simply let it go for another two years, without first checking around a little. As soon as I left the office and thought about some of his comments, I thought, "What normal mom WOULDN'T get a little worried about it?!"
Well, he's the doctor, not me, but *I* think it's something you should be concerned about and it's something worth seeking a second opinion over. Both the hospitals you mentioned are excellent places to take your kid.
I think you should probably wait to see what the forum doctor says, since he/she is FROM the Cleveland Clinic. That should be an indicator as to what you should do.
Best wishes, and if you have any more questions, feel free to ask...
I think you should probably wait to see what the forum doctor says, since he/she is FROM the Cleveland Clinic. That should be an indicator as to what you should do.
Best wishes, and if you have any more questions, feel free to ask...
THANK YOU! Yes, let's see what the Dr. says. I think these people at Cleveland Clinic really know their stuff.
Any dilation of the ascending aorta that is accompanied by pain needs to be carefully evaluated by an aortic specialist because symptoms trump size, regardless of what that size is. Any dilation of the sinuses seen by echo needs to be followed up with a CT or MRA. Echos cannot always visualize the entire Ascending aorta. A possible "tissue disorder" to accompany a dilated ascending aorta (the sinuses are the part of the ascending just "downstream" of the aortic valve) is a bicuspid aortic valve--1-2% of the population has a BAV and some studies show that up to 50% of those are accompanied by an aneurysm.
Thank you for your responses. I was so hopeful to get some kind of an opinion from the forum doctor as to whether or not I should be worried about all of this, given the actual facts. However, I still don't know! Does anyone else know if this sort of degree of dilation is a big deal?
New threads will probably be closed soon. If anyone has helpful info they'd be willing to share, I'm at ***@****. Thanks a lot.
faxmom -
In my opinion, this degree of dilatation *IS* a big deal, and you SHOULD be concerned.
More accurately, this degree of dilatation is big-enough of a deal for you to seek a second opinion very soon. It needs to be examined by a qualified pediatric cardiologist, preferably at either the Mayo or Cleveland Clinics or even a large hospital like Univ. of Minnesota, as soon as possible.
If she was MY daughter, I would:
1.) Get the soonest available appt. with a pediatric cardiologist - do some phone work to find out what's the soonest available, and make sure to emphasize that she's having symptoms and the size of her dilatation to get in quicker.
2.) Seriously reduce her physical exercise until the condition if evaluated by a good, solid cardiologist.
Again, others may differ; that's what *I'd* do.
Don't stress yourself out too much over it. Worry is useless. Action is fruitful. It's disconcerting, but with proper medical care, everything should be fine. If she complains about severe pain, take her to the ER, but besides that, schedule and go to a good cardiologist, and everything will be OK.
God bless.
Nick
In my opinion, this degree of dilatation *IS* a big deal, and you SHOULD be concerned.
More accurately, this degree of dilatation is big-enough of a deal for you to seek a second opinion very soon. It needs to be examined by a qualified pediatric cardiologist, preferably at either the Mayo or Cleveland Clinics or even a large hospital like Univ. of Minnesota, as soon as possible.
If she was MY daughter, I would:
1.) Get the soonest available appt. with a pediatric cardiologist - do some phone work to find out what's the soonest available, and make sure to emphasize that she's having symptoms and the size of her dilatation to get in quicker.
2.) Seriously reduce her physical exercise until the condition if evaluated by a good, solid cardiologist.
Again, others may differ; that's what *I'd* do.
Don't stress yourself out too much over it. Worry is useless. Action is fruitful. It's disconcerting, but with proper medical care, everything should be fine. If she complains about severe pain, take her to the ER, but besides that, schedule and go to a good cardiologist, and everything will be OK.
God bless.
Nick
I would also get a second opinion, the doctor is right, but but he has poor bedside manners, and should be relieved of his/her services. I would get another appointment, but this isn't a critical situation, so don't expect to be seen like tomorrow. It could happen, but not likely.
In other words, you have alot of time to deal with the situation. I'm talking in terms of months to years. There is no need to freak out right now. I don't believe they would be alarmed unless it was over roughly 4.0+ cm at her body size.
As far as the size of the aorta and pulmoary artery, they have things called z scores that calculate the appropriate size vessel measurments for the appropriate size body. We also measure the aortic root, sinus of Valslava and part of the ascending aorta to determine z-scores. There can be people who have Marfans while they don't outwardly have the typical Marfan like features. If you other cardiologist has any doubts, they'll refer you to a geneticist that can give you the skinny on the situation.
The aorta could rupture, but that could easily be years (20+) down the road, and this is at the very very earliest. I would also expect to be seen for an echo every year to check on the size of the aorta. She might get beta blockers (medicine) to take to help lessen the "load" on the aorta. She might have to take these for the rest of her life, but really that is getting ahead of things as they stand right now. As far as getting a CT or MRA like schmoomcgoo said, that probably won't happen either. Echo can generally see the area that is affected by Marfan's quite well. The echo would also be able to determine if the aortic valve is bicuspid or has three leaflets like it is supposed to.
As far as reducing her physical activity at this point, I wouldn't. Right now there isn't enough to make me think otherwise. She has a relatively long way to go before she gets into troublew aorta-wise. It might be a real possibility in the future (don't hide that from her), but for now, no.
Good night, and good luck.
In other words, you have alot of time to deal with the situation. I'm talking in terms of months to years. There is no need to freak out right now. I don't believe they would be alarmed unless it was over roughly 4.0+ cm at her body size.
As far as the size of the aorta and pulmoary artery, they have things called z scores that calculate the appropriate size vessel measurments for the appropriate size body. We also measure the aortic root, sinus of Valslava and part of the ascending aorta to determine z-scores. There can be people who have Marfans while they don't outwardly have the typical Marfan like features. If you other cardiologist has any doubts, they'll refer you to a geneticist that can give you the skinny on the situation.
The aorta could rupture, but that could easily be years (20+) down the road, and this is at the very very earliest. I would also expect to be seen for an echo every year to check on the size of the aorta. She might get beta blockers (medicine) to take to help lessen the "load" on the aorta. She might have to take these for the rest of her life, but really that is getting ahead of things as they stand right now. As far as getting a CT or MRA like schmoomcgoo said, that probably won't happen either. Echo can generally see the area that is affected by Marfan's quite well. The echo would also be able to determine if the aortic valve is bicuspid or has three leaflets like it is supposed to.
As far as reducing her physical activity at this point, I wouldn't. Right now there isn't enough to make me think otherwise. She has a relatively long way to go before she gets into troublew aorta-wise. It might be a real possibility in the future (don't hide that from her), but for now, no.
Good night, and good luck.
Wow. VERY helpful information. THANK YOU! I think we'll go ahead with the second opinion--mainly because the cardiolgist we saw was so short with us, and we left with a million questions. It sounds like we would have a ways to go before rupture would be a true worry--that's reassuring. My daughter is currently training to run a 10k with her gym teacher in late spring, and I was about ready to put the breaks on that. I would feel horrible if anything we were letting her do contributed to enlarging her aorta further. She loves competitive swimming and running, but I'd be more than willing to try to help her find other activities, if her health is being compromised. Thanks again.
Hi there. Do either of you know where I might find a simple to read "chart" that shows "normal" ranges/sizes of aortas and pulmonary arteries? I've looked around a little, with no luck. I'm at ***@****. Thanks again.
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.
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.
No, your earlier response was not at all too long. I totally agree with you, and I already have a call into my daughter's pediatrician for a 2nd referral. I'm hoping we can get into Mayo. I'm sure I'll be met with some resistance, since Children's Heart Clinic has such a great reputation. When he gave us the referral, he said that "all of the doctors are great over there." At the same time, I really don't think that was the case for us. So, hopefully, it will go okay. I also want to continue researching the condition and understanding the "norms", since I'm better at asking the right questions, etc., when I'm totally prepared and have a good understanding of the condition ahead of the appointment. Make sense?
The z-scores are generally calculated in a formula, but to be honest, I don't know what the formul is, just what it does and how it is used. I would agree that it would be ultimately easier for you and your daughter to let the pedi cardilogist to put things in the right perspective given all of the information. ?Not to say that you can't read a chart or do a formula, but over the years I've learned that you need to take in a multitude of factors to come up with a safe and reasonable approach to the situation. These are the things that pedi cardiologists ( and the adult ones too) know that the general public doesn't know, and that isn't readily available for easy consumption on the internet. I hope this helps some more.
Good night and good luck.
Good night and good luck.
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