Pediatric Heart Expert Forum
8 yr old son with moderate to severe aorta dilation
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8 yr old son with moderate to severe aorta dilation

Hi there,
My now 8 year old son was at a routine annual pacemaker check-up when it was discovered during his echo that he has moderate to severe dilation of the ascending aorta.  We are new to Colorado so this was our first visit to the cardiologist here. The cardiologist says she needs to do some more background checking to see if this is a change from his previous echo.  Upon speaking to our prior cardiologist he didn't note any previous issues with the width of the aorta, though he is sending a copy of the last echo we had done prior to moving so the new cardiologist can compare.  She said the dilation was +7. I don't really know what that means.
My son was born with 3rd degree heart block and has a mild vsd.  Before age 1 he had a PDA ligation, and at age 4 he had a pacemaker abdominally placed to manage the heart block.  Aortic dilation was not anything we had heard about or were expecting could be a problem.
Can you please advise what our next steps might be? And if surgical intervention is required, do kids who undergo this surgery usually go on to lead a full life without complications? Our doctor mentioned the possible use of beta blockers or replacing the root of the aorta but she won't have a firm recommendation until next week.  Which seems extremely far away.
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Children with small VSDs as their only cardiac lesion would typically NOT have aortic dilation. Nor would it be an issue with heart block and pacemaker placement.   It can be seen commonly when the aortic valve is bicuspid, so they need to look carefully at the aortic valve anatomy to see if it is normal or abnormal.  The issue with aortic dilation is "what is the risk or dissection or rupture?".  In patients with connective tissue disorders like Marfan syndrome, this is a real risk.  So your child should have a formal genetics evaluation if the aortic size is significantly abnormal.  Unfortunately, without seeing the report, I cannot assess how much dilation there is.  Another important issue is "what is the rate of change?" in the aortic size.  If the aorta is changing rapidly in size, that is a risk for rupture and might lead to a surgical intervention.  Your current cardiologist is correct in obtaining as many older images as possible to check the previous dimensions of the aorta.  Until that information is available, one cannot prognosticate on what this means in your particular case. Regardless, he would likely be restricted from competitve and isometric sports, as they can stress an already dilated aorta.
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Jeffrey R Boris, M.D.Blank
The Children’s Hospital of Philadelphia
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