Pediatric Heart Expert Forum
Double Aortic Arch w/ CoA of right arch
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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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Double Aortic Arch w/ CoA of right arch

Hello! I will be setting up an appointment to come to CHOP as soon as my daughter's medical records arrive there, which should be anytime this week.  At 6 months, she was diagnosed with a 4mm ASD. 3.5 years later, we were at her 4-year check up (echo) and found that the ASD had closed, but some type of arch anomaly was suspected and a murmur was still present.

A few weeks ago, she had an MRI and MRA and a double aortic arch was found. The left arch was atretic and created a vascular ring with tracheal narrowing. The right transverse arch was severely hypoplastic.  So a cath was recommended to measure pressures and this was done last Wednesday. The cath confirmed a CoA of the aorta. The hypoplastic segment of the right arch was 4.5 mm in diameter, whereas the lower portion of her aorta was 11 mm. Her pressure gradient was 30 mm HG (sedated). Aside from stridor, she has had no obvious symptoms of these defects, so we're still reeling a bit - though her blood pressure at her cardiologist's office was 123/75 for her 4 year check AND her 3 year check. It was always normal at her pediatrician's office, but in hindsight, it may have been taken from her left arm, not her right.

So - it's very difficult to find any information about CoA of the right arch and I can find NO information about the double arch and the CoA together. My daughter is extremely active (read: hyper). Until we can get our second opinion appt. set up at CHOP - are there any activity limitations or restrictions that we should be aware of? Dietary restrictions (i.e. no sodium, etc)?  I'm presuming since she has done well thus far, we are not looking at an emergency situation (this has not been mentioned by her treating cardiologists), but do we need to be on alert for anything?

Thank you in advance!
773637_tn?1327450515
Dear Sbogle,

It will be near impossible to find any information about coarctation of a right aortic arch or double aortic arch plus right arch coarctation.  However, unlike adolescents and young adults, the good thing is that children are quite good at self-limiting their activities.  If it feels uncomfortable, they'll stop.  Although I cannot say for sure what needs to be done without more information, I would say that there are no specific restrictions, precautions, or dietary issues that need to be done at this point.  Unless there are other specific cardiac problems that have not been diagnosed in her, she should be fine to await her second opinion.
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