Pediatric Heart Expert Forum
Double Aortic Arch/Digeorge possibly
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Double Aortic Arch/Digeorge possibly

16 mnth with Parainfluenza 3 months ago with ICU stay his airway was severly comprimised, during an stay in ICU the reintubated him which blocked up his tube and caused a cardiac arrest and he suffered severe brain damage from this via hypoxic eschemic insult, over the last 3 months after being told he would not walk/talk/see/hear etc he has done fantastically well and making abgreat recovery. Recent MRI tests revealed damage to basil ganglia region which affects movement and balance. all other area's appear to have redeveloped nicely## broncoscopy noticed indent last 3rd of his trachea  and a CT and MRI scan revealed a Douible aortic arch which we are waiting to see a cardiathorasic sergion in Leeds England next week.  I have a few questions about this.  the anesthetist could get size 5 ENT down no real issues apart from mild stridor on extubation.## Is this a simple procedure and one that has a good sucess rate of pulling through and what are the risks? Is it possible an operation may not be needed? this wouldnt have caused him to have the Cardiac arrest would it, as far as I know his heart itself is fine and he had an ECG which showed no issues.# Will his life expectancy be cut short

20% of children with this are a candidate for digeorge which makes me think, was if his reduced airway from the DAA that when he got flu it swelled up and did the damage OR may he had digeorge and it was his lack of immunity that comprimised his airway further and affceted him that badly. he has no other symtoms (symptoms) of digeorge really apart from when he was younger he was sick a lot but that resolved after a few months, he was also born with a very slightly bigger kidney but am told this has resolved itself, the only other thing to mention is when he was 4 months he got severe chicken pox but other than that he has been healthy and the perfect little man, he has no facial features of DGS and his calcium levels appear fine, no seizeures weight on fine. they also thing he has a thymus.
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773637_tn?1327450515
Dear Dave and Julie,

Early on as the embryo forms, there are a number of double sided arches.  Various parts of these arches disappear or become incorporated into other structures that eventually make up the aorta, pulmonary artery, and ductus arteriosus.  A double-aortic arch is seen when there is a remnant of the embryologic structures that make up the aorta.  The two sides of the arch surround the trachea and can compress it, causing respiratory distress.  This is most frequently seen in infancy.  However, in your son’s case, it appears that the combination of the compression and the internal airway swelling associated with the parainfluenza infection led to his respiratory problems and the subsequent discovery of this congenital anomaly.

As you found, it is associated with DiGeorge syndrome, or chromosome 22q11.2 deletion.  If a thymus was seen on his imaging studies, the likelihood that he has this chromosome deletion is lower (but not zero).  A genetic test will be needed to fully determine this, though.  Unfortunately, the manifestations of DiGeorge can be quite variable, including both their appearance as well as which organs are affected and how much, so I cannot fully predict whether he has it, or not.

Without having all of the information in your son’s case, I cannot say for sure, but typically repair of a double-aortic arch is a simple procedure, depending on the skill of the surgeon as well as the experience of the institution.  He should otherwise do well afterward.  That said, because his airway was externally compressed for so long, there is a chance that he has a fixed obstruction that may require relief later when he grows up.  In the end, this, as well as the neurologic outcome from the basal ganglia damage, will require observation over time.
5 Comments
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Avatar_m_tn
Thank you so much for your swift reply,we have also had calcium levels checked and all appears to be ok. We have had his lymposite count done and its fine but waiting for his T and B cell split which hopefully will be tomorrow. Is there a chance that surgical intervention is not always needed in all cases and is there ever an instance where it can't be operated on. My consultant not heart just general says it depends on where the vessels are located on the arch so you could have 2 on one side and 2 on the other or even in this case would a vascular ring split work? Sorry a lot of questions. We should no soon about digeorge but I would hope that if he had no immune at all he would be sick a lot more often.

On another matter whjat do you know about baclofen if anything, my son has been weaned off it due to no spasms for months but today after withdrawl (withdrawal) he has been all unsettled
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773637_tn?1327450515
There can be different configurations of the vessels, but, by and large, a true double aortic arch surrounds the trachea, and requires surgery.  The reason for this is that it remains a fixed external obstruction to the trachea, not only at rest, but also with growth, so it will impinge on it eventually if it isn't doing it now.

With regard to his baclofen therapy, you will need to discuss this with your neurologist.
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Avatar_m_tn
thanks agin for the reply, I was kinda hoping you would say that as his trachea grows then the surrounding Arch would grow with it.... the thought of surgery and the risk of losing him frightens me to death after all he has been through recently.

I was talking to a 28 year old lady the other day who has had a DAA and diagnosed from 6MO and never had surgery so was hoping for the same.
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Avatar_m_tn
hi there, we are waiting to see our Cardia surgeon in Leeds, can I just ask are most DAA acceptable for surgery, someone has mentioned about if vessels/branches are coming off both arches it is difficult to seperate/impossible to seperate as you wouldnt be able to cut and keep the branches - can that be true?
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