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Sudden Cardiac Death in a Young Athlete: Imaging Techniques to Evaluate the Etiology

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Highlights

  • Anomalous aortic origin of the coronary artery is a rare congenital heart defect.

  • Most patients are asymptomatic; however, some patients present with sudden cardiac death.

  • There are risks and benefits to different imaging modalities used for diagnosis.

  • Surgical repair is dependent on the specific lesion and patient lifestyle.

Abstract

Anomalous aortic origin of the coronary artery (AAOCA) is a rare form of congenital heart disease with varying implications. Although most of the population with this congenital heart defect may remain completely asymptomatic and never know of the diagnosis, the risk of sudden cardiac death among healthy children and young adults during or just after exercise is significant. A previously healthy adolescent female athlete presented to the pediatric intensive care unit after sudden cardiac arrest on the playing field. She was successfully resuscitated, and further imaging revealed a diagnosis of AAOCA. She underwent surgical correction of her defect without complications. AAOCA remains a rare and often undetected form of congenital heart disease based on the asymptomatic nature of the defect; however, with proper imaging, a diagnosis can be made to allow for correct treatment.

Introduction

Anomalous aortic origin of the coronary artery (AAOCA) is defined as a form of congenital heart disease in which both the right and left main coronary arteries arise from the same side of the aorta, or aortic sinus, either with one common origin or two separate origins (Brothers, Gaynor, Jacobs, Poynter, & Jacobs, 2015). The coronary artery that is arising from the incorrect aortic sinus will then typically have an intramural course within the aortic wall and descend to its proper location. The anomalous coronary artery can also take an interarterial course and lie between the pulmonary artery and aorta, or an intraconal course, and descend through the myocardium. Different variations of this congenital heart defect include anomalous aortic origin of the right coronary artery (AAORCA) arising from the left aortic sinus and anomalous aortic origin of the left coronary artery (AAOLCA) arising from the right aortic sinus (Figure 1).

The true prevalence of this congenital heart defect is unknown, simply based on lack of symptomatology among affected patients until they experience sudden cardiac death (SCD); however, studies have shown that approximately 0.1% to 0.7% of the population is affected (Brothers et al., 2015). In addition, studies have shown that the interarterial AAORCA is approximately three to six times more common than interarterial AAOLCA (Brothers et al., 2015). Of note, AAOCA is a completely different anomaly from anomalous left coronary arising from the pulmonary artery (ALCAPA), which is a form of congenital heart disease that typically presents at 3 to 6 months of life. Symptomatology of ALCAPA typically includes congestive heart failure and left ventricular failure.

The risk of SCD in those with AAOCA is greater during or just after exercise when cardiac output and myocardial oxygen demand are at its greatest. Myocardial ischemia because of impaired coronary blood flow through the anomalous coronary artery leads to ventricular arrhythmias and subsequently SCD. Impaired coronary blood flow derives from multiple factors including compression of the anomalous coronary artery itself because of its abnormal course, the typically small ostium of the anomalous coronary artery, and often the acute angle at which it arises from its anomalous origin. Active children and young adults are the most of those who experience SCD (Poynter et al., 2014). In young athletes in the United States who were previously healthy, AAOCA is the second leading cause of SCD because of undiagnosed congenital heart disease (Poynter et al., 2014). The purpose of this article is to present a case report of AAOCA and describe the current recommended imaging for definitive diagnosis to promote optimal management and outcomes.

Section snippets

Case report

A 14-year-old female with no significant medical history presented to the pediatric intensive care unit (PICU) after cardiac arrest while playing field hockey. According to witnesses and her parents, she had been playing field hockey and suddenly requested to leave the game, complaining of chest pain. She then collapsed and was noted to be pulseless, apneic, and with epistaxis. Cardiopulmonary resuscitation was immediately performed, and on arrival of the emergency medical technicians (EMTs)

Discussion

As discussed previously, it is difficult to determine the true number of people affected with this specific type of congenital heart disease simply because of the lack of symptoms. Because this type of congenital heart disease cannot be detected with a routine history and physical examination, the diagnosis is typically made after the patient experiences symptoms such as chest pain with exertion, syncope, dysrhythmia, and/or aborted SCD. In some cases, the diagnosis is made postmortem.

The need for automated external defibrillators

It is estimated that approximately 1 in 70 high schools through the United States will have someone experience SCD on their campus with about half of the victims being students and student athletes (Drezner, Toresdahl, Rao, Huszti, & Harmon, 2013). The availability of an automated external defibrillator (AED) at schools and athletic facilities has increased SCD survival to 80% versus 50% if the AED is brought from a responding EMT unit (Drezner et al., 2013). Unfortunately, barriers to AED

Conclusion

Although AAOCA is not a highly prevalent form of congenital heart disease, if undiagnosed or not properly diagnosed, patients may experience significant adverse consequences including SCD. Diagnosing AAOCA requires utilization of proper imaging techniques while taking into account individual patient factors as well as risks and benefits. TTE remains the initial imaging modality used in the diagnosis of AAOCA followed by either a cardiac MRI or a CTA to confirm the diagnosis.

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