DX: True Single Right Coronary Artery

[Proudmomma3xs]

Would love some guidance. My 14 yr old son has Chiari 1 malformation (just had decompression surgery 3/16/10), he also had a pin hole VSD that based on echos Friday and 2 yrs ago hasnt been a concern and is expected to close. (It can only be heard when he is lying down) He also has many marfan syndrome characteristics (inverted sternum mild, scoleosis very mild, rib anomaly, some hyperflexibility, 6'3" size 16 feet to name a few) but not the typical heart concern seen with marfan or facial features but many of the rest. Hes mild yet obvious skeletal anomalies seem to also fall under some of EDS (connective tissue disorder). He is seeing a geneticist in June. We have been told by a number of Chiarians that he seems to fit the bill for EDS with Marfanoid Habitus.

His docs, including his cardiologist are all intrigued/baffled by his case. Today was his follow up for the VSD he was diagnosed with "True Single Right Coronary Artery". He was actually dx with it two years ago but at the time he wasnt concerned by it at all. When I questioned his pediatrician he said it was a variation of "normal" and not a concern.  Now all of a sudden the docs tone changed and I am confused. I cant find much on this except that it is rare, (can't find a single thing that using his exact wordage "true single right coronary artery" esp when no other heart condition is present (Not sure if VSD fits that part). He said it can present two ways, one is fine (is he right??)and the other life threatening if the artery between. He is ordering additional testing. MRI with contrast or cat scan or catherization. I am wondering was he able to in way tell from the echo which it is more likely to be or is there no way to know and it is literally a 50/50 chance. HE didnt even talk about restrictions etc with either presentation.. Trying to find what % of those with this are non life threatening versus life threatening. And if it isnt life threatening are there any other concerns restrictions connected to it? At times he has complained of right chest pain and wondering if related or no connection at all since it is right side mainly. When he had his surgery he was on moriphine and his oxygen dropped to 79-82 one night for a bit he suffered sleep apnea issues on hospital soon as he tried to sleep he would gasp to breath and was placed on oxygen. The other day he said he was having trouble breathing but we think it was anxiety from going out after his surgery. He looked fine and was able to breath, it just hurt. His normal BP is 96/60. Yesterday 116/60ish (cant remember lower number) Would love to hear from anyone who has seen this or has experience with it or can explain this better to me. I think now that there are other things going on with him doc now sees this as a concern but why didnt he before??. I have been to multiple boards on the internet and sadly having trouble finding anyone with knowledge on this. I can find studies etc but not much else. :( Would so love some guidance!

TX!! Robin ~

I'll include a photo the doc drew for me. The top is normal heart he said he has one of the two below he said. Middle one being life threatening.

http://i238.photobucket.com/albums/ff240/boutiqueshoppes/DSC01917.jpg

[kenkeith]

Thank you for sharing.  I would not be able to constructively comment on any photo (diagram)that shows the abnormality.  

Usually when a diagnoses or an opinion is made, it is made on the basis of a differential analysis, meaning all possible conditions (signs and symptoms) that could indicate a serious situation has been discounted.

[Proudmomma3xs]

I just called his Ped and had them read me the report from two years ago ~ he said it was a benign variant.  It is my understanding from his apmnt Friday and ordering testing that to know if a concern or not that he has to do MRI/CTscan or cath to know so why did he say benign without testing to confirm??  Did he just guestimate? Very confused ~ TX so much!!    

[Proudmomma3xs]

TY so much for your post :)  That is actually a web link so you can copy and insert into your browser to view photo.  Doc drew the artery coming from the top of the AV, the artery that goes downwards is missing from his drawing.  Does this mean his is Anterior?  In general those with this, even if Anterior or posterior, are there higher risks with exertion?  I know any anomaly probably has risks but just curious if this is something that should be noted as a risk factor when exerting himself or if it really isnt if it doesnt go between.  Havent heard from his doc yet I assume it will be a few days til I do because he was consulting with other docs to decide the course of action.  I do have a sense of peace in knowing that finally the puzzle peices are beginning to fit with him and even though some things may be scary he is finally being understood so we can get him the help he needs.   Its the not knowing that is killing me.   He just returned today to school after being out for 2.5 months. Very hard to let him go, esp not knowing about this new concern and having just had decompression brain surgery.  TX again!  Robin ~    

[kenkeith]

Sorry but the forum's computer software is not set up to accept visual images.

The indecisions of the doctors  may be based on the congenital malposition or displacement abnormality of the RCA.  For some insight of the isolated single coronary artery, the anomalous origin of the right coronary artery (RCA) has originated from the left anterior descending artery (LAD). This variant practically never runs an interarterial course (between the aorta and the pulmonic artery) and is not known to be embedded within the aortic wall, and as a consequence, it is not known to be associated with any significant clinical syndromes. However, anteriorly displaced RCAs usually cannot be selectively imaged with conventional RCA catheters and thus may still pose a serious problem during diagnostic or therapeutic coronary interventions.

I'm not able to provide any information on how the condition relates to your son's situation or health, but from what you have stated the doctors have an understanding of the problem and will be able to provide the appropriate treatment.