My 5 year old's dilated aorta

[Mike C]

My son had a playground accident and was having some tests, since he needed to be screened for bav anyways, I asked them to echo.

it turns out his ascednign aorta is dilated. He is seeing a cardio at Childrens Hospital-Boston and we have an oppointmnet for his mother and I to talk to childrens hospital-marfan's expert. although its not marfans, its bav- i like to see how they treat it in marfans patients.

if anyone has any informaiton, facts, links, etc specifically as it relates to bav and aortic aneurysms please post or foward. I consider myslef to be very well educated on condition in adults, but want to make sure I dont miss anything in regards to kids.

z scores are as follows:

valve 1.65 zscore is 2.19
root 2.43 zscore is 3.19
ascedning 2.05 zscore is 2.12
arch 1.37 zscore is .32

cardio wanted to se him every three years for followup, talking with marfan guy who reviewed his records said he would act as cardio did but 1 year screenings.

 

[PJmomrunner]

I'm not sure I completely understand the z score concept. So his valve is 2.19 times the standard deviation larger than the mean? And likewise 3.19, 2.12 and .32 for the root, ascending, and arch respectively? I think it would valuable to know how his descending compares. I know there's some formula for predicting the size of the ascending based on the size of the descending. Is he a big guy?


Well, at least you know and you've got a baseline. I imagine it's pretty tricky to predict "normal" sizes as a little guy grows.

 

[aussigal]

I dont know what these "z" scores are either...

Not that it changes my answer anyway...I have 2 BAV sons both much older than your lad. My boys are on yearly follow-ups. The younger one has an enlarged Aorta and the other has a mild coarctation(narrowing). I think the Marfan experienced guy is probably your best bet for someone to manage his condition as long as you are comfortable with him and his assesments and recommendations.

My kids are on no restrictions. They are both aware that they have the condition and know that if they feel unwell or weird and there is a possibility of it being heart-related that they are not to ignore the symptoms and should rest or go get help. Basically they know its OK to rest when they are out being boys if they feel breathless instead of pushing on until they burst or collapse or something...hopefully this knowledge will keep them safe. They saw what I went through and I barely made it and dont want to have it happen to them.

You dont want to scare the kids but you do need to somehow instill a level of knowledge that will help them recognise when or if they are in medical trouble.

 

[Mike C]

Thank you all,

A z score is the number of standard deviations above/below what is expected for someone of same age, ***, etc. I dont fully understand myself but understand from some well respected docs thats its the number to key on.

We dont know descending numbers as he hasnt had a full ct scan or mri just echo and i think we probably wait until a little older. Its a good point and I have been comparing his root to his arch, which has a significant difference.

aussigal, not sure if your boys lift weights, but I would try to restrict them on that, not cardio or sports but just weightlifting. The other thing, their is a drug called losatran you may want to look into, its approved on market for blood pressure but has shown promising results in mice with dilated aorta's. Clinical Trials under way now for aorta piece. I need to do more research on it.

 

[Susan BAV]

Hi Mike -

I can imagine your worry here. And that must have been quite some playground accident; I hope your little guy is doing okay.

I don't have any concrete information to offer--mostly just heartfelt best wishes. But I do recall about a year ago a member, QueenVictoria, had a son (much older--late teens) with a sudden situation and she seemed to have done a lot of research. I think they suspected Marfan's but I don't recall if it was actually diagnosed. Perhaps searching her posts might be helpful?

Also, I know Lynlw has recommended a site--you may know she's been dealing with her son's heart issues since he was very young--but I don't quite recall the site; it's related to a child with genetic heart issues though and I understood that it might be a forum-type site similar to this one. If she sees this she'll probably post something helpful; otherwise maybe you could search her posts?

Take care. Best wishes.

 

[Curtsmum]

So sorry you are going through this with your son, sending hugs and best wishes to you and all the family

 

[briansmom]

Mike,
You're on the right path with your research. I'm sorry that I don't really have anything to add, we didn't discover my son's problem till he was on deaths door. I am a firm believer in knowledge - at the very least it allows you to maintain your illusion of control!

 

[francie12]

Mike,
We discovered my older son's BAV when his little brother sprained his ankle, and while big bro happened to be in the office the dr took a casual listen with the stethoscope. Subsequently, we found out that the sprained ankle son also has a dliated aorta, without the BAV.
I just wanted to mention that after the first couple of echos showing increasing dimensions (AO and LVdd), my older son was put on the drug, Lisinopril (at age 12) and it seemed to effectively slow down the progression. When you look at his yearly x-rays side by side, with the first couple showing his heart almost filling his chest cavity and the last couple looking much more normal it is dramatic. And there are no noticable side-effects. It is something you could talk to your dr. about. If our younger son continues to show increasing dimensions next year (beyond what is normal for growth of course), he goes on it too. I'm not a dr, but just thought I'd mention what happened with us.
Best of luck.

 

[Lynlw]

Hi Mike -

I can imagine your worry here. And that must have been quite some playground accident; I hope your little guy is doing okay.

I don't have any concrete information to offer--mostly just heartfelt best wishes. But I do recall about a year ago a member, QueenVictoria, had a son (much older--late teens) with a sudden situation and she seemed to have done a lot of research. I think they suspected Marfan's but I don't recall if it was actually diagnosed. Perhaps searching her posts might be helpful?

Also, I know Lynlw has recommended a site--you may know she's been dealing with her son's heart issues since he was very young--but I don't quite recall the site; it's related to a child with genetic heart issues though and I understood that it might be a forum-type site similar to this one. If she sees this she'll probably post something helpful; otherwise maybe you could search her posts?

Take care. Best wishes.

Hi, www.tchin.org is one of the groups with parents that are really knowledgible about CHDs.They have email list. There is a registration fee of I believe 20 dollars, but if money is a hardship they will work around that. They are one of the groups that are probably the most well known and alot of ped cards work with ect. and do alot to raise awareness.
I don't know much about Aortas, but will keep your son in my prayers. Boston Childrens is one of the best places for CHD. We took Justin there when we were looking for new doctors and if they were closer, probably would have kept them as Justin main center. They also have an Adult w/ CHD clinic which is great because when your son gets older, he won't have to go to a whole new set of doctors, which I think is a plus. Lyn

 

[aussigal]

Thanks for reminding me about the weight-lifting restrictions...I was very conscious of this and as soon as I knew I had an aneurysm I barely lifted anything heavier than a dinner-plate. Our boys dont do weights as a sport but their lives do involve lifting heavy stuff and being typical boys they are very competitive...I think its time for a little reminder about this subject., that its OK to lift but they shouldnt be straining themselves or they might burst.