How do you approach management of anomalous right coronary arteries?

My 11 year old daughter and husband have both been diagnosed with anomalous right coronary arteries originating from the left sinus of Valsalva (apparently genetic in our family). My daughter was an athlete until she was diagnosed. She is asymptomatic – it was a fluke that we found it. We have seen 3 cardiologists each with a slightly different perspective on how to proceed with her treatment. Basically, we can let her lead a normal, active life knowing she has about a 1 in 1000 chance she’ll have a cardiac event doing it; eliminate sports; or surgically correct it. My question to you is: what is the general recommendation you give an asymptomatic, athletic child with this abnormality? Do you have any protocol you follow?

I should mention that she has had a stress test and 2 ECG’s which were normal. Her echo and CTA just showed the anomalous artery, no other problems. There has been a difference in opinion as to whether it is intramural or not and how much compression there is at the ostium, but per the cardiologists here, there is no way to analyze the characteristics and determine which of these anomalies will cause an event. Is this in agreement with your findings, or have you found characteristics that pose a greater risk? Thank you.

Submitted by Kelly from Greenleaf, Wisconsin on 02/20/2014

Dear Kelly: We have a protocol. We do intracoronary ultrasound, both baseline and during Dobutamine infusion. It is a simple technique that allows more precise evaluation of the degree of compression. This is not a rare anomaly but difficult to make precise and sometimes arbitrary recommendations.