For Parents

Any symptoms during or immediately after exertion, such as chest pain, shortness of breath, fainting (syncope), feeling faint (near syncope) or palpitations are concerning. Also watch for symptoms that occur with exertion and do not subside with rest. Family history of an unexplained sudden death at an early or middle age is also of concern.

Make an appointment with the pediatrician to see if a pediatric cardiology evaluation is recommended. A transthoracic echocardiogram with Doppler color flow mapping may be indicated. If AAOCA is suspected, a coronary computed tomography angiogram and a stress cardiac magnetic resonance imaging may be required. In some cases, cardiac catheterization is necessary as well.

It’s safest if your child doesn’t participate in exercise or athletic activities until the evaluation with our cardiology experts is completed. Eventual recommendations will depend on the type of AAOCA he or she has.

For patients with anomalous aortic origin of the right coronary artery (AAORCA)

The newest guidelines from the American Heart Association and the American College of Cardiology report that if a child diagnosed with AAORCA has normal blood flow to the heart muscle, as determined by additional studies, they can return to highly competitive sports. Parents must know that there might be a slightly increased risk for sudden cardiac death. It’s encouraged that the athlete continues to have an exercise stress test once a year, although the approach may vary according to institutions and presence of symptoms. This is not currently our approach at Texas Children’s CAAP based on our data acquired thus far.

Some families choose to transition to lower intensity sports in hopes of decreasing the risk of sudden cardiac death, but the effectiveness of this is unknown.

For patients with anomalous aortic origin of the left coronary artery (AAOLCA)

AAOLCA is known to be a higher risk condition in certain individuals depending on the course of the anomalous coronary artery. In this case, careful evaluation with an expert cardiologist is necessary to determine the appropriate management and suitability for return to exercise or sports participation.

For those with AAOLCA, surgery is most often recommended even if asymptomatic, depending on the course of the anomalous vessel. This is typically done at age 10 or older.

For those with AAORCA, surgery is only recommended if your child is also experiencing concerning symptoms, there is evidence of decreased blood supply to the heart muscle or we have concerns with significant narrowing of the vessel. Often, patients with AAORCA don’t undergo surgery or have exercise restrictions.

Coronary translocation and unroofing procedures are the names of the most common surgical procedures for AAOCA. Coronary translocation surgery divides the abnormal coronary artery from its exit point and reimplants it to a more correct position. Unroofing procedures open a coronary artery that has an intramural course, meaning that it runs through the wall of the aorta.

Three months after surgery, a thorough evaluation is repeated with the same studies that were performed before surgery. If we have no concerns, children and teenagers are allowed to gradually return to athletic activities. It’s very important to build up tolerance to conditioning gradually after several months without physical activity and to be aware of any symptoms that may develop. Appropriate hydration is also essential for safe exercise performance.

If no surgery is performed, an annual follow-up with a cardiologist is recommended. If surgery is performed, your child will need close postoperative monitoring, and then yearly evaluations.

Frequent evaluations after surgery are necessary to look for signs of complications that may be related to the heart valves or the heart muscle itself. Risk of these complications are reduced when the surgeons have extensive experience. Follow-up monitoring will continue throughout your child’s life.

There is no cure for AAOCA, but, when managed by a dedicated cardiology team, your child can live a full and healthy life.

There is a possible genetic link, but the exact genes are unknown currently. Families with more than one child affected have been reported and have been seen in our program. Consideration to screening family members should be given and will be discussed at the time of the evaluation.

We recommend that parents take an advanced CPR class where they learn both how to perform CPR and how to use an AED. We also recommend that you make sure there are AEDs readily available at any sporting events in which your child participates.

Look out for signs and symptoms during or following exertion such as chest pain, dizziness and fainting. If your child has a history of asthma, be aware that some AAOCA symptoms can mimic an asthma attack.

If you have concerns that your child might have AAOCA, talk to your pediatrician. The Texas Children’s Coronary Artery Anomalies team is also available to you as a resource.

There are no formal recommendations or guidelines regarding personal ownership of AEDs. Some families choose to acquire one for their child. This can be discussed further with your cardiology provider.

For Coaches

All athletic trainers should be CPR certified and always have access to an AED.

Those with AAOCA may have chest pain, shortness of breath, heart palpitations and/or fainting during or immediately after exertion. However, signs and symptoms may be absent in at least half of people who experience sudden cardiac arrest due to their AAOCA.

All activities should be stopped, parents should be notified and an appointment should be made with the athlete’s primary care provider. All trainers and coaches should be aware that the athlete should not participate in any physical activity until cleared by a doctor.

Aside from having the proper equipment readily available, an emergency action plan should be prepared at your facility. This plan should be practiced with all staff that works with athletes to ensure that everyone is aware of how to proceed in case of an emergency.

  • Stop all activities.
  • Have someone call 911.
  • Someone trained in CPR should initiate the CPR protocol with use of AED.
  • Notify the athlete’s parents.
  • When EMS arrives, be sure to inform them of the circumstances around which the arrest occurred and any known symptoms and medical conditions.
  • Instruct parents to make an immediate appointment with their health care provider.
  • Do not permit the athlete to participate in any exertional activities until cleared by a health care provider.