For Parents

What signs and symptoms should I be worried about in my child with AAOCA?

Any symptoms during or immediately after exertion, such as chest pain, shortness of breath, fainting (syncope) or feeling faint (near syncope), or palpitations.

Symptoms that do not subside upon resting after exertion.

Family history of unexplained sudden death at an early to middle age.

What should I do if my child is having signs and symptoms?

Make an appointment with the pediatrician to see if pediatric cardiology evaluation is recommended.

A transthoracic echocardiogram (TTE) with Doppler color flow mapping may be indicated.

If AAOCA is suspected, a coronary CTA and a stress CMRI may be required. In some cases, cardiac cath is necessary as well.

Can my child continue to play sports prior to the evaluation or after the diagnosis?

It is safest if your child does not participate in exercise/sports activities until the evaluation with cardiology experts is completed.

AAORCA (anomalous aortic origin of the right coronary artery):

The newest guidelines from the American Heart Association (AHA) and the American College of Cardiology (ACC) report that if a child diagnosed with AAORCA does not have changes due to lack of sufficient 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 SCD. It is encouraged that the athlete continues to have an exercise stress test once a year, though 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.

If a child is at increased risk, a change from a highly competitive sport to a lower intensity sport could decrease the chance of SCD, but this is yet unknown.

If possible, a change in position within their sport could be advised.

AAOLCA (anomalous aortic origin of the left coronary artery):

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

When is surgery recommended?

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 10 years of age or older.

For those with AAORCA, surgery is only recommended if they are also experiencing concerning symptoms, there is evidence of decreased blood supply to the heart muscle, or there are concerns with significant narrowing of the vessel. More often than not, patients with AAORCA do not undergo surgery or exercise/sports activities restrictions.

What type of surgery is typically performed?

Coronary translocation and unroofing procedures are the most common surgical procedures for AAOCA.

Please, see section above for figures and descriptions of these techniques.

How soon can they return to sports after surgery?

At three months after surgery, a thorough evaluation is repeated with the same studies that were performed prior to surgery. If there is no concern, the child/athlete is allowed to gradually return to sports activities. It is very important to build up tolerance to conditioning gradually after several months without physical activity, and to be aware of any symptoms that might develop. Also, appropriate hydration is essential for safe exercise performance.

What does the future look like?

If no surgery is performed, an annual follow-up with a cardiologist is recommended.

If surgery is performed, the patient will need close post-op monitoring and then yearly evaluations as detailed above.

Possible post-op complications include: valvular regurgitation, left ventricular dysfunction, chronic pericarditis, pericardial effusion and possible small infarct. The risk of these complications are reduced when the surgeons have extensive experience.

Follow-up monitoring is lifelong. There is no cure for AAOCA.

Is this condition genetic?

There is a possible genetic link, but it is unknown at this time. Families with more than one child affected have been reported and are present in our CAAP.

Consideration to screening family members should be given and will be discussed at the time of the evaluation.

How can I be better prepared to deal with complications?

Learn CPR and how to use an AED.

Make sure there are AEDs readily available at sporting events/competitions.

Look out for signs and symptoms during or following exertion such as chest pain, dizziness and fainting. If there is a history of asthma, be aware that symptoms may occur with AAOCA that mimic an asthma attack.

Talk to the pediatrician if there is concern the child may have AAOCA. The Texas Children’s CAAP team is also a resource.

Should we get a personal AED?

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

What equipment do I need to have readily available?

All athletic trainers should be CPR certified and have access to an AED at all times.

What signs and symptoms should I watch out for?

Those with AAOCA may present with chest pain, shortness of breath, heart palpitations and fainting during or immediately after exertion. However, signs and symptoms may be absent in at least half of those prior to presenting with an SCA.

What to do if an athlete is experiencing these symptoms?

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

How can I be prepared in the event of sudden cardiac arrest?

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

What should I do in the event of an athlete having an eminent sudden cardiac arrest?

  • Stop all activities.
  • Assess need to initiate CPR or use AED, and have someone call 911.
  • If the AED indicates a shock is needed, proceed with the shock. If no additional shock is indicated by the AED, resume CPR immediately.
  • Notify 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 a health care provider.
  • Do not continue sports/exercise activities until cleared by a health care provider