Double Aortic Arch in Siblings

[daveandjulie123]

Hi There, Ive posted on here before its been a great help.

We have 2 sons, My youngest who is now 3 had a Double Aortic Arch but we didnt know until he had a Cardiac Arrest at 14 Months old and he is now disabled, doing very well though. His website is www.williamstonestrustfund.com he had surgery in 2010 September and is coping pretty well, an element of tracheamalacia but seams to handle coughs and cold with help of atrovent and brown preventer and the odd dose of oral steroids in emergencies. Yes hes doing well. At the time before surgery he was tested for various syndrones mainly Degeorge (22/11 deletion) and all was negative.

My question is my eldest son who is now 5 has just been diagnosed with Asthma, he just coughs he doesnt have any wheeze or excersize induced, he has a lot of allergies too. sometimes DAA is passed off for asthma but i think thats when they have lots of stridor, wheeze and recurrent URT infections, he doesnt have any of that, no vommiting no other signs.

This got us thinking, DAA is so rare, less than 1% of all CHD but i cant find any info about it being genetic, can you advise me as he was never tested for the same condition, should we have or would you thought he would have been symptomatic by now? its just this recent asthma that got us wondering which is mainly night time coughing.

All the best

Dave

[Marie M Gleason, M.D.]

Your question about occult vascular rings and the diagnosis of 'asthma" is a good one.  There are occasions where a patient has a relatively "loose" vascular ring and symptoms don't start to crop up until they are older.  Given your past experience with your younger son, I think that any knowledgeable physician would understand your concern and be willing to get your older son assessed in some way.  The simplest thing would be to have a Chest Xray evaluated.  As you know, in vascular rings, the dominant arch is on the right side, and that can often be picked up on a routine chest xray if you are looking for it.  Otherwise an echocardiogram should be able to pick up arch anatomy and sidedness.  Statistically it is unlikely that both children have vascular rings, and the genetic link would have to be extremely slim.  Much more likely that he actually has reactive airways.  Good luck!

[Marie M Gleason, M.D.]

It sounds like the basic echocardiogram was normal.  The only piece of information not obtained from that was the sidedness of the aortic arch.  So hopefully the CXR will settle that issue.  If it is a left sided aortic arch, all should be well.  As far as the ECG is concerned, a prominent R wave in V1 in a 5 yr old is usually non-specific and age related.  If the echo shows no right heart enlargement or RVH it is not anything "real" to be worried about.  So once you get the CXR report hopefully you can relax.

[daveandjulie123]

Hi There, well we have taken Alfie to the Cardioligist and all is not clear, I dont understand really what the guy is saying to be honest. He had an ECG which showed R wave in V1 this im told could be a sign of Right Ventricular Enlargement (RVH) however he then did an echo and could find no evidence of any enlargement, see below.. He has now sent him for a chest X ray to totally rule out Double Aortic Arch. Please Please Help as Im confused.. he has no symptoms of RVH.. He is 5 years old..

HELP !!!

LETTER FROM CARDIOLIGIST

Thank you for your letter dated the 2nd May 2012 about this five-year old boy.


I gather that there is a distressing story in regard to his three-year old brother who had a congenital heart defect.  He got a cold and then suffered a respiratory arrest, and later a cardiac arrest with some hypoxic brain injury.
He was then found to have a double aortic arch, which was causing an obstruction to his trachea and presumably oesophagus, and this was operated on at Leeds General Hospital, following which he did well.

The course of events here has definitely had an effect on Alfie’s parent’s perception of any symptom or illness.  Alfie was recently diagnosed with asthma and is allergic to forms of grass, silver birch and dog hair, but his parents were worried in case his mild respiratory symptoms represented any underlying major unknown defect.

He had a normal pregnancy and delivery, he was 9lb 11oz at birth but he did not feed well for a couple of weeks, but then things picked up.  He now takes a steroid inhaler twice a day and Cetirizine antihistamine.

In terms of the effects of his brother’s illness, Alfie has developed some mild behaviour issues eg., when William’s situation is discussed in depth.  However, he otherwise appeared to be a healthy and active young child with good humour.

The venous pulse was normal, there was no radiofemoral delay and the heart sounds were normal with normal splitting of the second heart sound.  The ECG showed sinus rhythm and there is a dominant r-wave in V1.  This may be partly due to electrode placement but is not explained otherwise.

The echocardiogram shows that left and right ventricular function appeared good.  The right-sided structures do not appear enlarged or hypertrophied.  The LV diastolic dimension was 3.8cm, and the end systolic dimension 2cm.  The aortic root measures 2cm, and the left atrium 1.6cm.  The overall left ventricular ejection fraction is about 70%.  The aortic valve was tricuspid and as far as I could tell with rather poor views scanning through the upper rib cage, the aortic arch appeared single.

As I was not able to get perfect views of the aortic arch, and because of the dominant r-wave in V1, I sent Alfie for a chest x-ray at the Alexandra Hospital.  The results of this should be back within a couple of days.
From my point of view Alfie seems a healthy child with a normal looking heart.  Hopefully the chest x-ray will be completely normal to help confirm this.

Yours sincerely

Adam Fitzpatrick

Consultant Cardiologist & Electrophysiologist