Which treatment plan for Single right conoray artery is best

[Proudmomma3x]

My 14 yr old w/True Single Right Coronary Artery.  You've been so helpful, I wanted to ask another question. The angle it arises has been reviewed w/Dr. Cheitlin (Anomaly specialist) His anomaly is slightly different then typical one associated w/sudden death. All agree that it is most likely a lower risk.  However limited data on his anomaly is available making it very hard for us to have peace of mind in monitoring alone.  Although risk is lower the risk is still there none the less since it still does go between the great arteries. We're also getting 2nd opinion from Boston.  

The 2 options put on the table are bypass surgery (Quote from surgical meeting) "First of all the bypass of the artery would be done with the internal thoracic artery, a picture of which we got at the catherization anticipating that it could be used down the line.  He pointed out however that if the internal thoracic artery graft is put in then the left main coronary would have to be ligated since competitive flow would cause internal thoracic artery graft to shrivel up & become of less use."  

The less invasive option is to put in a defibrillation device.  (we have been told this is a bandaid, not correction) It would help somewhat but still would not prevent massive heart attack should it occur.  We love the idea of the less invasive surgery however after hearing the risks associated with this "off label" approach (since he isnt symptomatic) we arent so sure.  The risks of infection, inability to do MRI's (he has chiari and basiliar invagination) so needing to do CT's instead, rejection, and misfires has us concerned. The risks of the surgery we know is high (death or complications) but once done & recovered he should do very well & the problem is fixed. Where as the defib device isnt correcting & being lifelong w/risks I am very concerned.   We have opted to treat, not monitor and would love some thoughts on which is better for long term safety weighed with the risk.  Thx! Robin ~

[Jeffrey R Boris, M.D.]

Hi, Robin,

I've pasted several URL's that link to articles discussing your son's coronary anomaly.  The better way to search for it is for "single coronary artery," and look for the documents that discuss the right coronary arising from the left coronary artery (NOT the left coronary ostium).  Hope that helps.

http://eurheartj.oxfordjournals.org/content/13/12/1637.abstract
http://www.springerlink.com/content/yvp03utekp13wbjp/
http://www.cardiothoracicsurgery.org/content/5/1/49
http://www.ncbi.nlm.nih.gov/pubmed/9367100?dopt=Abstract&holding=f1000,f1000m,isrctn

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[Proudmomma3x]

One more question :) Do you know of any sites, links, articles etc online that talk specifically about his version/anatomy of the anomaly.  I cant find anything with "true" SRCA.  I am not great with the terminology since I am not in the medical field but everything I find seems to talk about it arising from a different location then his.  I think if I found just once case like his it would help, but sadly I cant :(  Thank you so very much!!  Robin ~  

[Proudmomma3x]

Thank you SO much for your post ~ I am so greatful to all you have done so far in helping us!!   As you can tell I am desperately searching for the right answers.   When I search online for his DX the only thing that comes up are my posts on the message board :(  His cardiologist is helping us get another opinion from Boston Childrens Hospital.  He too has shared that the limited data on his specific anomaly makes it very hard to know what is best.  I am so looking forward to hearing what he has to say :)  I would def be willing to come down to PA also if that is what I need to do for the best options for him :)  I do plan to get copies of the catherization and stress test for Dr. Deitz for when we see him in October. So can def get them for other opinions too if it helps and by all means I have no problem what so ever talking to you specifically.  :)  Just let me know how I can reach you if so.  I am a scared mom and will do what ever I need to do to ensure he is safe and sound :)  If it helps this is what he wrote most recently to describe the specific anatomy of the anomaly. This is the description given to lastest doc at Boston Childrens Hospital.. ..The specific anomaly, that was discovered and has been proven by catherization and selective coronary angiography, is a True Single Right Coronary Artery from which originates the left main coronary artery. This in turn proceeds backwards and to the left between the great vessel roots after which it bifurcates into a normal circumflex and anterior descending coronary. So this is not the origin of the left coronary from the right sinus of Valsalva directly ~ a known risk factor for sudden ischemic events."  Thanks again!  Robin ~

[Jeffrey R Boris, M.D.]

Dear Robin,

Without the studies to review, nor your son to evaluate, nor you to talk with specifically, I am not able to assess risk, interventions, or potential outcomes for your son.  This is a very specific question to both your son and to you, and is much more involved than the forum that this message board provides.  In order to give you the best answer possible, I would recommend having the information reviewed in its entirety along with having a complete evaluation of your son and a discussion with you.

[Proudmomma3x]

Forgot to mention that he is a swimmer and left handed which doc said effects the specific location slightly the device would be added :)  TX!!