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Coronary Artery Anomalies Program

 Anomalous Aortic Origin of a Coronary Artery

Normal anatomy of the origin of the coronary arteries

The coronary arteries are vessels that arise from the aorta (the biggest vessel in the body that delivers blood from the heart to the body).There are usually two main arteries: the left and right coronary arteries. The right and left coronary arteries arise from one of three outpouchings of the aorta called sinuses of Valsalva.  

 Anomalous aortic origin of a coronary artery (AAOCA) is a congenital cardiac abnormality (present at birth) in which the origin or course of the coronary artery that arises from the aorta is atypical.

AAOCA is the second leading cause of sudden cardiac death (SCD) in young athletes in the United States.

People with AAOCA may be asymptomatic, however, in up to 50% of cases or more, and the first manifestation of the disorder can be sudden cardiac arrest (SCA) or sudden cardiac death (SCD). Others may develop cardiac symptoms leading to this diagnosis upon medical evaluation.

The figure to below shows the most common types of AAOCA we evaluate and manage in our program, among other types of anomalies, such as myocardial bridge.

The left hand panel shows AAOCA that have an interarterial (in-ter-are-teer- ee-al) course. The interarterial course is a condition in which the coronary artery travels between the two great arteries – the aorta (the artery that takes blood to the body) and the pulmonary artery (the artery that takes blood to the lungs to receive oxygen).

The right hand panel shows anomalous coronary arteries that have an intramural (in-tra-mur-al) course, in addition to an interarterial course. The intramural course is a condition in which the first part of the coronary artery has grown within the aortic wall and may increase risk for ischemia, which is decreased blood flow to the heart muscle.  

Another type of AAOCA is the one that includes an intraseptal course, which means that the anomalous coronary travels inside the heart muscle for a specific length before surfacing the heart. Patients may be asymptomatic (without symptoms) but some may experience symptoms. 

During your child’s clinic visit, the cardiology provider will go over the type of AAOCA your child has.

Occurrence

AAOCA has a low prevalence in the general population, estimated as 0.06-0.9% for anomalous right coronary artery (AAORCA) and 0.025-0.15% for anomalous left coronary artery (AAOLCA). Boys are three times more likely than girls to have AAOCA.

AAOCA from the opposite sinus of Valsalva accounts for 14-17% of all cardiac-related deaths in youth. AAOLCA is considered more lethal than AAORCA, being responsible for up to 85% of SCDs related to AAOCA. Most cardiac events appear to occur in individuals between 10-30 years of age. Yearly occurrence of SCD among young athletes varies widely, and is estimated between 1 in 43,800 and 1 in 200,000. Most SCDs from AAOCA occur during or just after physical exertion.

Sports commonly associated with SCD include the following, among others:

  • Basketball
  • Soccer
  • Track and field
  • Swimming
  • Cross-country running
  • Football

Causes

AAOCA is a congenital heart condition. Congenital is defined as present at birth.  As with most congenital heart defects, a specific cause cannot be identified and may be multifactorial.

Clinical Presentation – Signs & Symptoms

About half of patients with AAOCA present without symptoms and the diagnosis is suspected on cardiac imaging (heart ultrasound or echocardiogram) performed for another reason. However, those who are symptomatic may present with:

  • Chest pain during or immediately following exertion
  • Heart palpitations during or immediately following exertion
  • Shortness of breath/difficulty breathing during or immediately following exertion
  • Dizziness during or immediately following exertion
  • Fainting during or immediately following exertion
  • Cardiogenic shock
  • SCA (sudden cardiac arrest)
  • SCD (sudden cardiac death)

The most common approaches (at the discretion of the surgeon and cardiologist) are exercise restriction and surgical procedures, depending on several factors following careful evaluation with experts and studies performed.

Exercise Restriction

Exercise restriction is used to decrease the risk of SCD, however it is unknown if this truly has an impact. Moreover, this can be difficult:

  • Children are competitive in nature.
  • There is still a possibility of SCD with minimal activity that would not necessarily be prevented with exercise restriction.
  • Exercise is both a physical activity and a way to connect with peers. Asking a child to refrain from activities can create psychological and emotional consequences.
  • In the long term, exercise is important for overall cardiac health, and exercise restriction may increase the risk of cardiac events in the future.

Our Approach

Our team at Texas Children’s Hospital looks at each patient as an individual and formulates the best plan of care/recommendations. This may be surgical intervention, or clinically monitoring the patient regularly in the cardiology clinic, with the goal of returning to full exercise activities, without restrictions, whenever possible.

Surgery

Surgery is also used to decrease the risk of SCD, however it is yet unknown if it truly has an impact. The benefits of surgery are better defined in the setting of the higher risk AAOLCA, but less so in the setting of the lower risk AAORCA. The presenting symptoms and the studies performed are essential to help in the decision-making, a process shared between patients/families and the CAAP multidisciplinary team.

The type of surgical intervention is determined by the anomalous coronary anatomy, though the two most commonly used techniques are coronary artery reimplantation (relocation) to the correct sinus and unroofing procedure.

Coronary Reimplantation

The abnormal coronary is divided at its exit point from the aorta and reimplanted into the correct sinus of Valsalva.

Unroofing Procedure

Unroofing is used when there is a long intramural segment of the coronary. The wall between the aorta and coronary artery segment that is traveling through the wall, known as the intramural segment, is opened in order to prevent coronary artery compression. Unroofing the intramural segment also aims at placing the ostium of the coronary in the correct sinus of Valsalva.

Ostioplasty

Ostioplasty is used when there is a fibrous ridge around the coronary ostium causing obstruction. The fibrous tissue is removed, improving the size of the coronary ostium. This technique may be associated with the unroofing procedure.

Coronary Artery Bypass Graft Surgery

This technique has been used to treat adult patients with AAOCA. However, this technique is not frequently used in young children and adolescents because of competitive flow between the bypass graft and the native coronary artery, which may result in failure of the bypass graft.

Results

All patients continue to be followed longitudinally, regardless of undergoing surgery or not (if not indicated).

For those followed clinically, repeating studies is usually not needed for at least 3-5 years from the initial assessment, unless clinical concerns arise, and the great majority of patients are allowed to continue with exercise/sports activities with no restrictions. 

For those undergoing surgical intervention, a complete evaluation, as done on the initial presentation, is repeated at 3 months after surgery. This is to determine readiness to return to exercise/sports activities with no restrictions. Continued longitudinal follow up is also recommended and repeating studies is usually not needed for at least 3-5 years. There have been rare reports of SCD after undergoing AAOCA surgery, possibly related to incomplete resolution of the anatomical issues at surgery.

A very small subset of patients may have a high-risk anatomy of the AAOCA that makes surgical intervention very challenging, with no optimal options or carrying a high-risk of complications. These patients may be offered a specific medication and exercise restrictions from exercise/sports activities.

Follow-up Care

The CAAP at Texas Children’s Hospital has developed a clinical algorithm to standardize the evaluation and management of patients with AAOCA. This algorithm is refined on Quality Assurance multidisciplinary meetings every 1.5-2 years when the data acquired is revised meticulously and changes to the algorithm, if needed, are made.

Non-Surgical follow-up:

  • Pediatric cardiology follow-up every year
  • ECG every year
  • Echocardiogram every two years (optional)
  • Exercise stress test every 2 years (optional)
  • Stress cardiac MRI every 3-5 years (optional)

After surgery follow-up:

  • Postoperative Short-term Follow-up may include: 
    • 1 week: cardiovascular surgery follow-up
    • 1 month: cardiology visit with ECG, echocardiogram
    • 3 months: cardiology visit with ECG, CTA and stress cardiac MRI. If cardiac cath was performed as part of the evaluation prior to surgery, it will be repeated again at this time. Exercise restrictions may be lifted after third month visit if studies are acceptable.
    • 6 months: cardiology visit with ECG
    • 12 months: cardiology visit with ECG 
  • Long-term Follow-up 
    • Pediatric cardiology follow-up every year
    • ECG every year
    • Echocardiogram every two years (optional)
    • Exercise stress test every 2 years (optional)
    • Stress cardiac MRI every 3-5 years (optional)

Lifestyle Changes

  • Patients awaiting surgical intervention or who refuse surgical intervention are restricted from exercise/sports activities.
  • Patients undergoing surgical intervention cannot exercise until 3 months after surgery, after which they can return to exercise/sports activities as long as they meet the following requirements: 
  • Patients with low risk lesions that do not warrant surgical intervention are allowed to continue exercising without limitations.
  • Physical activities and exercise is important to cardiovascular health.  Our aim is for our patients to maintain physical activity throughout their lives. 
  • They are asymptomatic
  • All the test findings are acceptable/negative

Quality of Life

  • Patients and families affected with this diagnosis often experience increased anxiety and this may alter their lives. Texas Children’s Heart Center has psychology support for our patients. All patients in the CAAP program are referred to psychology as part of our program.
  • Resuming exercise activities might be an important goal to achieve normalcy following a diagnosis of AAOCA, whether undergoing surgery or not.
  • Providing education and information about emerging data related to this condition may positively impact patients and families and continues to be our impetus to acquire prospective, longitudinal data through our Registry.

Psychological Adjustment

A diagnosis of AAOCA brings significant anxiety and stress to patients in families. Some of the reasons include uncertainty of outcomes long-term, risk of SCD after surgery, safety of continuing to exercise with no surgical intervention, uncertainties on the best options for management, restriction from exercise/sports participation, among others.

Therefore, our CAAP has included an experienced psychologist who meets with patients and families to provide help in coping with this new diagnosis. The hope is that this encounter will facilitate the expression of sentiments and identification of tools to better deal with AAOCA and as well as other coronary anomalies.

Community Engagement and Support

The National Coronary Anomalies Foundation (NCAF) was created by families affected by coronary anomalies and is a 501C non-profit organization. 

More information to come in the near future. 

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