Pediatric Heart Expert Forum
BAV with Dilated Ascending Aorta
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Questions in this forum are answered by pediatric cardiologists, cardiothoracic surgeons and anesthesiologists from The Children's Hospital of Philadelphia. This forum is for questions and support about pediatric heart problems, symptoms and topics such as heart murmurs, palpitations, fainting, chest pain, congenital heart defects (including management and intervention), fetal cardiology, adult congenital cardiology, arrhythmias and pre-participation athletic screening.

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BAV with Dilated Ascending Aorta

Healthy 7 yr old with z-score 3.6. Ascending aorta went from 2.38cm to 2.5cm in 2 year. BAV is functioning normally. LV dimension is in upper limits of normal for BSA which is .89m2.  No LVH...everything else is normal. Cardiologist advised no heavy lifting or breath holding exercises. How careful do I need to be? Could you help me understand this....

Is going from 2.38 to 2.5cm a significant change? From time to time he complains of chest pain, throbbing, is this a concern? He also has asthma. At what point is surgery required? How much of a concern is the dilation at this stage? Is 3.6 a high z-score? Is there any way to predict when surgery is required...1 yr, 10yrs from now? Any other information would be helpful.
Thanks.
Tags: aortic dilation with bicuspid, aortic dilation, bicuspid aortic valve
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Ascending aortic dilation is common in patients with bicuspid aortic valves, even those whose valves function normally. It likely represents an "aortopathy" or abnormal tissue within the wall of the aorta that predisposes it to stretching and is potentially weaker than normal.   A normal z-score is 0, and upper normal is 2.  Mild dilation is (roughly) a z-score of 2.1-3.0; moderate dilation 3.1-4.5 and a z-score over that is severe dilation.  So your child's ascending aorta is moderately dilated (common).  What the cardiologist follows is the rate of change, ex: increasing more than 1 cm in a year, or significant changes in z-score (up 1).  In general we don't have to do anything with these except restrict isometric activity and contact sports as there is likely some weakening of the aortic wall that is prone to further stretching and potential tearing (dissection) with high amounts of stress.  Surgery for the aorta generally occurs at aortic dimensions > 5 cm.  Often the aorta never reaches a level for a surgical intervention, but it does need serial follow up.  As is the case in most children, your child's chest discomfort is likely of a non-cardiac nature, but you should discuss it further with your cardiologist.
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