Awareness Needed: Anomalous Aortic Origin of a Coronary Artery

October 12, 2017

5 comments

By: Thomas Kimball, MD

We’ve all read about the rare stories in the news where a teen collapses on a court or field from sudden cardiac arrest (SCA). As parents, they shake us to our very core. For cardiologists like me, they haunt us. We wish we could have done something to prevent it, especially when it happens in our hometown.

While hypertrophic cardiomyopathy is the leading cause of SCA in teens, there is a second-leading cause, which is not as well known. Because of this, I am asking for help in spreading awareness for it. In some cases there are warning signs, but they are subtle.

ANOMALOUS AORTIC ORIGIN OF A CORONARY ARTERY: DEFINITION

This rare congenital condition is called anomalous aortic origin of a coronary artery (AAOCA). It’s sometimes referred to as coronary artery anomaly for short. The simple explanation is that it happens when the coronary arteries arise from the wrong location leading to potential restriction of blood flow to the heart. Here’s a more thorough explanation: The heart has two coronary arteries (a left and a right) and the aortic valve has three leaflets to it. With AAOCA, the left coronary artery comes off the right leaflet, or aortic sinus, which causes it to course between the aorta and pulmonary artery. This can cause a squeezing issue of the coronary artery particularly during exercise.

For non-athletes this anatomical mix-up may not become an issue. It becomes problematic in middle school, high school and college, when athletes are capable of producing sudden bursts of exertion.

Here’s how: When we exercise, we need to provide more blood to our muscles, legs and arms. To do this, the heart has to pump more blood to the body. So during intense exercise, the heart and the blood vessels arising from it (the aorta and pulmonary artery) will dilate to eject more blood. When there is an underlying AAOCA, intense exercise and dilation of the aorta and pulmonary artery compression of the coronary artery can occur, much like a nutcracker, causing sudden cardiac arrest.

WARNING SIGNS OF AAOCA

Unfortunately, and in many cases, the first warning sign of this condition is sudden cardiac arrest. In a small number of cases, teens will have chest pain during exercise or dizziness during exercise before the SCA event.

POTENTIAL DETECTION

This is one of the reasons why the State of Ohio mandates all high school athletes receive a health screening. Twelve of the screening questions are heart related, and two could potentially catch AAOCA, if further tests are conducted. The questions ask if the athlete has had any chest pain during exercise or unexplained dizziness during exercise. If yes, the physician will refer to cardiology, where we would potentially conduct an echocardiogram. We may explore further with other cardiac tests, if warranted.

Depending on the type of coronary artery anomaly found, open-heart surgery will likely be performed to correct it. Athletes can return to their sports after surgical correction if postoperative testing is normal.

Because there are people out there with this condition and don’t know it, it’s hard to predict how many people have it. It’s estimated to be around .1 – .2% of the population.

WHAT PARENTS CAN DO

You might be wondering what you can do as a parent. First, check in with your athletes frequently. How are they feeling during practice and games? Are they having any chest pain or dizziness during exertion? What differentiates AAOCA, versus typical chest pain in teens, is chest pain during exercise. If your teen is having chest pain while at rest, it is not AAOCA.

Second, advocate for your school to have AEDs available and unlocked. Even better would be for the coaches to have them on the sidelines. If CPR and an AED are administered within three to five minutes of a sudden cardiac arrest, chances of survival are 56%.

Lastly, please share this post with your friends and family. Even though anomalous aortic origin of a coronary artery is rare, the symptoms are catastrophic, especially in athletes. More awareness is needed to help prevent the resulting symptoms of this silent condition.

To learn more about our Coronary Artery Clinic, which treats this condition, please visit our website or call 513-636-4432.

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Topics:

Heart Conditions

About the Author: Thomas Kimball, MD

Tom Kimball, MD, is a pediatric cardiologist and Medical Director of the Heart Institute at Cincinnati Children’s. He started the Coronary Artery Clinic, in which he is also the Director, because he has been witness to one too many teens collapsing from AAOCA. He loves talking to kids about athletics and physical activity and has ran 63 marathons in 44 states.

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Comments

Jules October 12, 18:18
Dr.Kimball~ Is there any kind of pre-screening available for this? Would an EKG/echo/ or "Stress Test" pick this up?
- Thomas Kimball, MD Author October 13, 04:34
  Hi Jules, An initial screening test would be an echocardiogram. However, this is expensive and generally not recommended in asymptomatic patients. If a patient has fainting with exertion or chest pain with exertion, an echo would be warranted.
Rajiv March 04, 13:04
Dr. Kimball - shared this amongst my friends and colleagues. Thanks for posting. Given the catastrophic nature both the American Heart Association and Insurance groups should figure out a way to optimize the cost of one time screening for all sports kids cutting out profits for a great cause
- Thomas Kimball, MD Author March 06, 10:41
  Mr. Sengupta - Thank you for your post. I agree that the conditions can have catastrophic consequences. Over the last few years, I’ve witnessed groups, including our own Heart Institute at Cincinnati Children’s, perform screening clinics and programs at either reduced costs or even gratis. The problem is sustainability not only in terms of cost but also in terms of the number of people needed to conduct large scale screening programs. Added to this is the less than perfect sensitivity and specificity of screening tests resulting in the possibility of false positives or even worse false negatives. And finally, the decision to screen or not to screen is, ultimately, a societal issue – specifically what is society willing to pay for detecting the number of children that can be saved by screening. With breast cancer, for example, society has deemed it appropriate that the cost of a mammogram is worth the cost of saving a number of women from that awful disease. At this point, unfortunately, the same cannot be said for large scale athletic pre-screening in children and adolescents.
Belle March 22, 19:07
I suffered from shortness of breath for a very long time after my daughter was born in 2007 and was misdiagnosed with asthma in 2014. this was after seeing a cardiologist who stated my heart was okay following a stress test. After being on steroids for a year, the pulmonologist stated he could not treat me for asthma any longer as the treatment was not working and he felt I did not have asthma. I moved on to another medical team in 2016 and a contrast CT scan was used to identify my anomalous RCA. By-pass surgery was recommended, no mention was made of an unroofing procedure. I had the by-pass done in 2016 and felt better for about a month before my symptoms returned. My cardiologist only said oops and sorry. I am about to have an unroofing procedure and reading these blogs have been enlightening and extremely helpful to me. I am now 50 years old and have no plaque or blockage in my arteries, I have a very healthy heart. I should not have had the bypass, my blood flow was fantastic. Everyone should do more research on their condition and ask for alternative solutions. I was informed this was the best course of action and no alternate solution was offered. It would have saved me a lot of pain and suffering. Once diagnosed, my doctor asked that I do not read up on the condition as it is frightening and I must not be panicked by the information out there. But reading about the condition helps patients understand what options are available to them. Because of my fear of going under the knife again so soon after the by-pass surgery, I was placed on drugs to slow my heart and open up my arteries. They helped me manage the symptoms but I could not exert myself and my fatigue is excruciating. My unstable angina was less frequent. Thank you so much for this information. I also wondered if anyone can have a normal action packed life after the unroofing procedure. It would be great to know.