Pediatric Heart Expert Forum
dilated ascending aorta
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dilated ascending aorta

Hi

My son 12 year old  now, was born with vsd,  when he was one year old he was diagnosed with subaortic stenosis and double chamber right ventricle .within 2 months he had an open heart surgery and got all corrected.  For the past 11 years at his annual check ups, doctor said everything is fine and no heart murmur and he is growing up active and fine.But last week after his annual echo his cardiologist informed us it shows dilated ascending aorta.and it was not there all this time.could it be because of his growing age?what are the other reason that caused this change?  Is there any chance that it will remain the same with out furtherdilation without any treatment? i would like to know all the possibilities in future.Also precautions I should take?He is a very good basketball player and very active child.could it be something that won't create any further problems to him? he had 2 strepthroat infections last month and had amoxicillin and cephalaxin,does it have anything to do with the change?
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773637_tn?1327450515
Dear Ambj,

In reading the information about your son, I can’t give you a complete answer, as I don’t have all the information that I would need to be able to make a determination as to what is happening and why the aorta is dilating.  However, I can tell you a bit about aortic dilation in general.  

First of all, I do not believe, with the available medical literature at this time, that the aortic dilation is associated with the streptococcal throat infection.  Streptococcal pharyngitis, especially when untreated, is associated with acute rheumatic fever, which causes a number of systemic findings throughout the body as well as the potential damage of the aortic and/or mitral valves.  Your description does not include anything to suggest that.  As well, I am not familiar with abnormal dilation of the aorta being associated with “normal” body growth.

It is unclear to me how dilated the aorta is and if there are any other associated risk factors.  One of the increasingly seen associations with aortic dilation is the presence of a bicuspid aortic valve.  This is when the second valve on the left side of the heart (between the left ventricle and the aorta) has two leaflets instead of the normal three leaflets.  It is unclear why these two are associated, but there is research going on in that field to try to find out why.  This finding should be able to be diagnosed by an echocardiogram (cardiac ultrasound).  Bicuspid aortic valve is the most common congenital heart defect, occurring in 0.5-2% of the general population.

Another association is the presence of Marfan syndrome, a genetic abnormality associated with very tall stature, certain physical features, including sunken chest, spinal curvature, hyperextensible joints, certain eye abnormalities, and several other findings, and heart findings, including mitral valve prolapse (abnormal closing of the mitral valve, the first valve on the left side of the heart) or aortic dilation.  More research has demonstrated a possible biochemical cause for this, and there is a ongoing study that is assessing two different medications in the slowing or stopping of aortic dilation.  The diagnosis of Marfan syndrome should be able to be made by clinical evaluation, echocardiographic findings, and, if necessary, genetic testing.  

There are familial aortic aneurysm syndromes that are associated with several different genetic abnormalities.  Knowledge of the family history of this as well as specialized genetic testing, as indicated, can help to diagnose this.  However, this is not particularly common.

Finally, we are realizing that there are certain congenital heart defects that are associated with dilation of the aorta, including tetralogy of Fallot, truncus arteriosus, and transposition of the great arteries.  These can occur, even after surgical intervention.  We can also see this after certain other types of heart surgeries, including an operation called the Ross procedure.  The reason for this is not clear, although it may be related in some way to the abnormal processes going on in bicuspid aortic valve or with Marfan syndrome.

At this point, your son will need continued cardiology surveillance, including routine history, examination, and echocardiography to look at the size of the aorta and to assess its growth.  It is not clear whether the medications that are being studied in the prevention of aortic dilation in Marfan syndrome are effective for aortic dilation associated with other clinical syndromes, such as those listed above.  It’s also not clear, if there are no other associated problems, whether the aorta will continue to dilate or whether it will stay the same relative size.  The literature seems to support the notion that the rate of aortic dilation associated with an otherwise normal trileaflet aortic valve without any other heart problems is slower than that of a bicuspid aortic valve.  Certainly, you should feel comfortable with the information that you are getting from your cardiologist.  If this continues to be confusing to you despite the information that you are getting, it is entirely reasonable to get a second opinion.
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The Children’s Hospital of Philadelphia
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