Pediatric Heart Expert Forum
what is the long term outcome
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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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what is the long term outcome

hello
my 9 years old son had an echo. test recently it was order by his doctor after she heard a murmur during his yearly check up.
his echo. shows that he has a bicuspid aortic valve with dilated root valve. Annulus is 1.9 cm +2.7 z score.
aortic Sinus is 2.5cm +2 z score. Ascending aorta 2.3 cm +3.5 z score.
his blood pressure is very good both in arm and leg. as well the oxygen level and heart rate are normal. there is no aortic stenosis or insufficiency.
he weights 57 lb and about 52 inches tall, very active and healthy- and never showed any sign of health problem.
the only thing his doctor recommended is to do a yearly Echo test to monitor his heart and for him to stay away from contact sport like football, which is very head for him to do since he loves sports.
my  question is from the resolts of the echo. is this a prograssive aortic root dilation and what is the long term outcome of his condetion. als is there any thing can be done at this age to correct his aortic valve.
thanks
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Dear Macbi,

The aortic valve typically is trileaflet, or has 3 cusps.  In bicuspid aortic valve (BAV), there can be 3 cusps with two of them fully or partially stuck together, or there can be only 2 cusps.  BAV can be associated with development of aortic valve stenosis (obstruction), insufficiency (leakage), and prolapse, as well as aortic root dilation.  BAV occurs in about 0.5 to 2% of the general population, making it the most commonly seen congenital cardiac defect.  The majority of people with BAV have a normal life, and many don’t even know that they have it.  However, they do require lifelong surveillance to ensure that they do not develop the complications listed above.  Over the long term, there is a good chance that the aortic root will continue to dilate.  

Your son’s echocardiographic numbers do demonstrate aortic dilation.  Whether he will require surgery for this depends on whether his aorta dilates rapidly or whether it gets to be larger than 4.5 to 5 cm in diameter.  Surgery can remove the dilation.  However, it never makes the valve or the aorta perfect.  I say to my patients that aortic valve disease is something you have for life in one way, shape, or form.  The goal, therefore, is to keep a surgeon out of the chest for as long as possible.  At this age and from his present condition, based on what you are telling me, neither I nor any surgeon would do anything to the valve.  It will not make the valve “better”, nor will it change the likelihood that the valve will have complications in the future.  We do know that the connective tissue that makes up and surrounds the valve is abnormal, and will thus be at risk for abnormal changes through his life.  That said, without seeing your son or his studies, I cannot say for sure what his needs will be.  

In the absence of moderate or severe complications at this point, most people would let your son be active, such as participating in aerobic exercise, like running and soccer.  There is concern that isometric activities (anything that involves exercise with straining, such as football, weightlifting, and wrestling) may accelerate the damage to the valve and the aorta, although we don’t know this for sure.  I tend to err on the side of being conservative, though, and not allowing isometric activities in those patients with BAV and aortic root dilation.
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The Children’s Hospital of Philadelphia
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