Tracheal stenosis after ASO for TGA

[sal2121]

My son, now 10 yrs old had his ASO at 2 days old for D-TGA IVS, normal recovery with mild Mitral value regurgitation and aortic root dialation(moderate).  He has always had gi reflux, treated with meds, not always well.  He was treated for years for asthma without much relief.  He started complaining it was hard to breath, all the time, about 1 year ago (after a big growth spurt)  He is just under 5 feet tall and is 105 lbs.  After numerous tests it was determined his aorta is compressing his trachea (seen on 3D CT scan). it has been classified mild to moderate but does not warrant surgical correction.  When his heart is really pumping wouldn't the pulsating aorta compress the trachea even more?  

At what point would surgery be considered?  His FEV1 is less than half of what is expected per function testing.  He becomes short of breath very easy now and doesn't want to do anything anymore, physically.  It was determined his lung volume is 25% higher than average and he uses 125% energy consumption during exercise testing.  

My concern is he is still growing but his airflow isn't.  He is having trouble now what will it be like for him when he is over 6 ft tall?  Should I take him to another pulmo doctor or insist his cardiac surgeon go in an pin back his aorta?

[sal2121]

Thank  you for your response, we have indeed  ruled out all other  causes, the compression was discovered  on ct performed while investigating his abnormal pulmonary function test results.  The compression is classified mild to moderate while at rest.  He was scoped a few months ago and some minor webbing was removed at that time.  It was determined he could "live with it" as far as the compression goes but that is not what I am seeing.  I realize that with everything he has been through we are lucky to even have him here, but I am hoping his quality of life can be improved from what it is currently.   I am going to take him to Mass General for Children, here in Boston for a second opinion.  Just to make sure we have covered all the bases.

[Marie M Gleason, M.D.]

Tracheal compression by the aorta or one of its branches is a very uncommon condition but it does occur.  It can happen with structurally normal or abnormal hearts.  The indication to do a surgical aortopexy (as you said "pin" the aorta to the chest wall to get it off the trachea) depends upon how well one can prove that the patient's symptoms are truly due to this and not to something else.  If you have documentable pulmonary function abnormalities that worsen with exercise and visible narrowing of the airway on imaging studies, then surgery may in fact be a reasonable step.  one notation: after aortopexy not all patients feel better, hence the slow response to intervene surgically.  If your son's symptoms are problematic enough, then certainly get a second opinion from another pulmonologist and/or cardiologist that can be presented to your son's surgeon.  Good luck.