A Hidden Threat: Anomalous Aortic Origins of the Coronary Arteries in Athletes

Abstract

Purpose of Review

Anomalous aortic origins of the coronary arteries (AAOCA) are a primary cause of sudden cardiac death in athletes. This review will detail the epidemiology, pathophysiology, and risk stratification of AAOCA, while also highlighting return-to-play considerations for athletes.

Recent Findings

Sport pre-participation cardiovascular screening methods lack sensitivity and specificity in the identification of AAOCA. For the symptomatic athlete, clinicians must maintain a heightened clinical suspicion for AAOCA in order to proceed with appropriate cardiac imaging and functional assessments. Anomalous origin of the left coronary artery with an interarterial course is considered high-risk and requires sport restriction until surgical correction. In contrast, risks associated with anomalous origin of the right coronary artery are controversial, thus management and sports eligibility decisions may incorporate principles of shared-decision making.

Summary

Management options for athletes with AAOCA are complex, requiring a comprehensive clinical evaluation. While advances in multimodality cardiovascular imaging and physiologic functional assessments have improved AAOCA risk stratification, best practice treatment strategies for some AAOCA subtypes remain uncertain. As such, clinical management and sport eligibility decisions require an individualized approach. Future prospective data will guide optimization of treatment strategies for athletes with AAOCA.

Introduction

Although anomalous aortic origins of the coronary arteries (AAOCA) are uncommon within the general population [1], AAOCA in athletes represents a common cause of sudden cardiac death (SCD) [2,3]. Athletes identified with AAOCA also present clinical management challenges based on the underlying anatomy and potential risks of downstream morbidity and mortality. As illustrated in the case vignettes, athletes with AAOCA may present with varying degrees of symptoms across the age spectrum. In addition, lack of effective screening methods presents significant barriers to the identification of AAOCA during the pre-participation cardiovascular exam [4,5]. Because of the wide range of anatomic variation present in cases of AAOCA [1], determining risk and appropriate clinical management strategies may lack certainty. Despite technologic improvements in cardiac imaging [6], a high degree of complexity remains with the risk stratification of differential AAOCA phenotypes. The assessment of AAOCA requires a detailed approach and shared risk discussions with the athlete to achieve an appropriate balance between mitigating the risk of SCD [7,8], surgical options [9], and the athlete’s desire to continue competitive sport participation [10,11]. Indeed, AAOCA represents a hidden threat for the sports cardiologist, always a consideration in the evaluation of an athlete and a clinical conundrum associated with numerous challenges from diagnosis to treatment. In this review of AAOCA, we will discuss clinically relevant anatomic variants, epidemiology, and imaging modalities relevant to the diagnosis and risk stratification of athletes with AAOCA. We will also detail considerations for return to competitive training in athletes with AAOCA.

Coronary Artery Anatomic Variation and Definitions

Nomenclature and categorization of normal coronary anatomy and phenotypic variations differ throughout the medical literature [12]. Generally accepted normal coronary anatomy consists of the left coronary artery originating from the left sinus of Valsalva and the right coronary artery (RCA) originating from the right sinus of Valsalva [1]. Normal variants, such as the presence of three coronary ostia, occur when the conal branch originates from the aorta separately from the RCA or when the left circumflex artery originates separately from the left anterior descending artery [12,13]. Anomalous coronary anatomy is present in <1% of the general population [13,14]. In addition to AAOCA, other categories of coronary artery anomalies include anomalous left coronary artery arising from the pulmonary artery, coronary myocardial bridges, and coronary artery fistulas.

AAOCA is based on the origination of a primary coronary artery from the opposite sinus of Valsalva, i.e. an anomalous origin of the left coronary artery (ALCA) originating from the right sinus of Valsalva or an anomalous origin of the right coronary artery (ARCA) originating from the left sinus of Valsalva. The type of AAOCA is further categorized based on the proximal to distal course of the anomalous artery. There are five courses that have been previously described: pre-pulmonic (anomalous course anterior to the pulmonic valve), retro-aortic (anomalous course posterior to the aortic valve and aorta), transseptal (also called subpulmonic), interarterial, and retrocardiac (anomalous course posterior to the tricuspid and mitral valves) [14]. Of these subtypes, the interarterial course, defined as the anomalous coronary artery situated between the aorta and main pulmonary artery, is the most malignant and believed to represent the primary AAOCA phenotype associated with SCD [14–16].

AAOCA Prevalence and Risk of SCD in Athletes

AAOCA prevalence data in the general population varies due to differences in case definitions and study methodology [1]. One of the earliest autopsy studies of 18,950 cases reported a prevalence of 0.3% [17], a value similar to a recent comprehensive review of 77 studies and >1 million patients. In this recent review, Cheezum and colleagues reported an AAOCA prevalence of 0.44% based on invasive angiography studies and 0.70% based on noninvasive studies [14]. In addition, prevalence of interarterial ARCA was 0.23% compared to just 0.03% for interarterial ALCA [14].

AAOCA is one of the leading causes of SCD in young athletes (Table 1) [8,16,18]. In the largest autopsy study of SCD in athletes to date, Maron and colleagues evaluated 1,866 athletes over a 27-year period. In this study, AAOCA was the second leading etiology underlying SCD, responsible for 17% of cases with an identifiable cardiovascular cause of death [8]. However, an important limitation of the reported prevalence was the exclusion of presumed cardiovascular causes without a precise diagnosis. In a more contemporary analysis of SCD in National Collegiate Athletic Association athletes, Harmon and colleagues found that AAOCA was the most common structural pathology, present in 14% of sudden deaths [19]. Among military personnel, reports of AAOCA associated SCD are even higher based on data from Eckart and colleagues, in which 33% (N=21) of 64 cases were attributed to AAOCA [20]. While the prevalence of ARCA is higher overall compared to ALCA, ALCA is more commonly identified in cases of SCD [2,8,15,16,20–22]. Due to this observation, ALCA is generally accepted to present a higher risk phenotype compared to ARCA [23]. The stable incidence of SCD attributed to AAOCA over time highlights the inherent challenge of identifying and risk-stratifying AAOCA in athletes. Current screening methods, including a comprehensive history and physical exam, screening 12-lead electrocardiogram (ECG), and trans-thoracic echocardiography (TTE) all lack the appropriate sensitivity and specificity to reliably exclude the presence of AAOCA in athletes [16,18,24].

Table 1.

Select Studies Examining Coronary Artery Anomalies and Sudden Cardiac Death in Athletes

Study	Population	Study Period	Key Findings
Harmon et al. 2014 [19]	45 cases of SCD in NCAA athletes	2004 – 2008	CAA was identified as the most common identifiable cause of sudden death. 5 (14%) of 36 cases with a determinable cause of death on autopsy died from CAA.
Eckart et al. 2011 [27]	902 cases of SCD in active military personnel	1998 – 2008	13 cases of AAOCA were identified overall. Of 298 deaths in <35 year-olds, 12 (4%) were due to AAOCA.
De Noronha et al. 2009 [26]	118 cases of SCD in athletes from the United Kingdom	1996 – 2008	CAA was identified as cause of death in 6 cases. Mean age of death due to CAA was 15.8±6.2 years compared to 27.9±12.5 years in the total cohort.
Maron et al. 2009 [8]	1,866 cases of sudden death in athletes from the United States	1980 – 2006	Of 690 confirmed cardiovascular deaths, AAOCA was the second most common cause of death, after hypertrophic cardiomyopathy, with N=119 (17%) cases. 65 cases of ALCA and 16 cases of ARCA were identified.
Eckart et al. 2004 [20]	126 military recruits with non-traumatic sudden death	1977 – 2001	Of 62 deaths due to cardiac causes, 21 (34%) were due to AAOCA. All 21 were interarterial ALCA. Pre-mortem symptoms were noted in 11 (52%) individuals.
Corrado et al. 2003 [28]	55 athlete and 245 non-athlete cases of SCD in ≤35 year-olds from the Veneto region of Italy	1979 – 1999	7 (13%) athletes had SCD due to AAOCA compared to just 1 (0.4%) non-athlete with SCD. AAOCA was the condition with the highest risk of sport-related SCD (RR: 79, 95% CI 10–3564, P<0.001).
Basso et al. 2000 [2]	27 athletes ≤35 years old with SCD and AAOCA identified from American and Italian athlete registries	N/A	23 athletes had ALCA and 4 had ARCA. All athletes died either during or immediately after exercise. 10 (37%) athletes complained of cardiac symptoms before death.
Burke et al. 1991 [25]	34 cases of exercise-related SCD and 656 cases of nonexercise-related SCD	1981 – 1988	4 (12%) exercise-related SCDs were due to CAA. CAA was more common in exercise-related SCD than nonexercise-related SCD (12% [N=4] vs. 1.2% [N=8], P=0.002)

AAOCA: Anomalous aortic origins of the coronary arteries; ALCA: anomalous left coronary artery; ARCA: anomalous right coronary artery; CAA: Coronary artery anomaly; NCAA: National Collegiate Athletic Association; SCD: sudden cardiac death

There are consistent findings taken from athletic SCD studies in the context of AAOCA [2,20–22,25–27]. First, the temporal association between intense exercise, either during or just after, and risk of cardiac arrest is evident based on multiple autopsy studies [2,20–22,25–27]. Burke and colleagues studied 690 SCD cases and identified AAOCA as the cause of death in 12% (4 of 34 cases) of sports-related deaths compared to only 1.2% (8 of 656 cases) in non-sports-related deaths [25]. In a study of 27 athletes with SCD from Basso and colleagues, all 27 athletes died during or immediately after exertion [2]. Second, risk of SCD is much higher in athletes compared to non-athletes, increased 79 times higher in athletes [28]. While the quantification of risk has been described among youthful athletes, risks among older athletes remain uncertain. Atherosclerotic coronary artery disease is the most common cause of SCD in athletes >35 years old [29,30], but the incidence of SCD due to AAOCA in older athletes is unknown. While it is possible that prior selection of high-risk AAOCA phenotypes limits the prevalence of malignant AAOCA in older athletes, SCD due to AAOCA still occurs in this population and remains relevant in those with and without established cardiac risk factors [9,21,23].

AAOCA Pathophysiology

The underlying pathophysiology associated with an interarterial AAOCA course is controversial, although several mechanisms have been proposed [31,32]. It is generally accepted that pathologic anatomic deformation of the coronary ostia and proximal portion of the coronary artery, including an acute angle takeoff [33], slit-like coronary ostia orifice [22], and intramural aortic course [34–36], leads to cross-sectional narrowing of the proximal portion of the vessel and represents a high-risk SCD feature [37,38]. These anatomic alterations are more prevalent in the interarterial AAOCA phenotype. From a functional standpoint, a ‘scissor-like’ physiologic effect has been proposed in which coronary artery compression between the great vessels occurs during times of high cardiac output (e.g. exercise), leading to myocardial ischemia and potentiation of malignant ventricular arrhythmias [15]. However, this hypothesis has been refuted by results from a study that utilized intravascular ultrasound (IVUS) imaging and demonstrated that the proximal portion of the interarterial coronary artery, closest to the aortopulmonary interface, lies intramural within the aortic wall [1,36]. Both underlying pathophysiologic hypotheses support the concept that abnormal proximal coronary anatomy predisposes to myocardial ischemia. This predilection for ischemia provides rationale linking the interarterial AAOCA course with high levels of exercise and corollary increased myocardial oxygen demand [2,20,21,25,28].

Several important in-vivo IVUS studies from Angelini and colleagues further support the hypothesis that underlying ischemic mechanisms are associated with a proximal, ovoid intramural coronary course with accentuated compression of the artery during systole with exercise [39–41]. In addition, prior data suggest that interarterial and intramural AAOCA consistently display varying degrees of hypoplasia and lateral compression [41,42]. These intramural segments also demonstrate subtle physiologic changes, detectable by IVUS, when pharmacologic stress is applied mimicking exercise hemodynamics [43]. However, limitations associated with pharmacologic challenges and the lack of correlation between coronary stenosis severity and clinical manifestations should be acknowledged [35]. Inducible ischemia associated with AAOCA is likely a dynamic process as demonstrated by the inability to reliably document ischemic changes during exercise stress testing [2]. Lee and colleagues analyzed predominantly symptomatic patients with AAOCA and found that changes in fractional flow reserve rarely decreased during stress, reinforcing the concept that ischemia associated with AAOCA is likely transient, not consistently reproduced with exercise or provocative testing [44].

AAOCA Clinical Presentations

Athletes with AAOCA may complain of a multitude of clinical symptoms with varying severity. Exertional symptoms that could be representative of myocardial ischemia are extremely concerning and include chest tightness that resolves with rest, dyspnea on exertion, exercise intolerance, dizziness or lightheadedness, or syncope during or just after exertion [45]. However, the absence of symptoms is unreliable as a discriminatory factor based on prior important data indicating that symptoms are absent in approximately half of AAOCA cases [2,20,21]. In the same study of young military recruits previously discussed from Eckart, of 21 identified SCD cases due to AAOCA, 52% (N=11) of subjects previously reported symptoms of chest pain, syncope, or dyspnea [20]. In the previously discussed study from Basso, only 37% (10 of 27 cases) of athletes who experienced SCD due to AAOCA complained of chest pain, syncope, dizziness, or palpitations [2]. Most important in this landmark study, none of the 6 athletes who had exercise stress testing prior to SCD had ischemic changes. As such, SCD is unfortunately a frequent initial presenting symptom of malignant AAOCA. As part of the risk stratification algorithm for AAOCA, exercise ECG testing alone is not a reliable risk adjudicating test. Functional assessments a part of exercise stress testing should be considered in the evaluation of newly diagnosed AAOCA [23].

The recent development of large AAOCA registry data has allowed for improved characterization of clinical presentations by prospectively capturing symptoms in living patients [31,46]. In a contemporary, prospective dataset of 163 patients with AAOCA (15% ALCA and 71% ARCA), Molossi and colleagues reported that exertional symptoms were present in only 21% of cases and non-exertional symptoms were present in an additional 20% of cases [46]. Notably, the presence of exertional syncope predicted high-risk pathology (OR 15.8, 95% CI 1.3–185.4) [46]. In a second registry of 198 patients from the Congenital Heart Surgeons Society, the most common presenting symptom was exertional chest pain in 24% of patients, followed by chest pain at rest in 15% [31]. In both of these registries, patients were asymptomatic in approximately 50% of cases, mirroring the 50% prevalence of symptoms from prior autopsy studies [2,20,21]. With the benefit of prospectively obtained data, it is affirmed that AAOCA lacks consistent symptomatology and is more often identified incidentally in the absence of effective screening strategies [45].

AAOCA Diagnosis: Imaging Modalities

Echocardiography

TTE is a rapid, low-cost, and widely available imaging modality capable of identifying coronary ostia and proximal coronary artery anatomy. With proper technical operation, TTE is generally able to identify both coronary ostia in >90% of cases (Figure 1) [40,47–49] and the addition of color Doppler enables further delineation of the proximal intramural AAOCA course [50]. However, there remain significant limitations with routine use of TTE. In a study of 159 patients, Lorber and colleagues observed poor correlation between institutional reports and “expert” echocardiogram reviewers, highlighting the need for standardized AAOCA TTE protocols [51]. The ability to consistently identify coronary origins with TTE also relies on experienced and technically skilled sonographers [52]. Further, additional technical limitations may be present as a consequence of body habitus or chest wall anatomy that may impair adequate coronary visualization [52,53]. As such, TTE is not considered the standard of care for evaluating suspected AAOCA [23]. However, because athletes commonly have TTE performed for other indications, there is an opportunity to evaluate for normal arising coronary ostia in a convenient cost-effective manner that limits potential radiation exposure. This strategy is recommended by current American College of Cardiology (ACC) and American Heart Association (AHA) guidelines [54].

Fig. 1. Examples of echocardiography and CCTA images of the coronary ostia and arteries.

A). Transthoracic echocardiographic (TTE) parasternal short axis image of normal right coronary artery (RCA) and left coronary artery (LCA) origins from the appropriate sinuses of Valsalva. B). TTE short axis image with color Doppler to confirm normal location of the LCA origin and left anterior descending artery. C). Coronary computed tomography angiography (CCTA) of an anomalous RCA arising from the left sinus of Valsalva with a malignant interarterial course and acute angle takeoff. D). CCTA 3-dimensional reconstruction of an anomalous LCA originating from the right sinus of Valsalva with a benign pre-pulmonic course.

Computed Tomography

Coronary computed tomography angiography (CCTA) has a class I indication for evaluation of AAOCA and is considered the gold standard, first-line modality for noninvasive coronary imaging in the clinical setting (Figure 1) [23]. In practice, CCTA is increasingly utilized for non-invasive coronary evaluation, accurately depicting coronary artery anatomy in 3-dimensions [6]. In addition to rapid scan times and relatively low cost, CCTA also provides comprehensive characterization of specific anatomic details beyond the capabilities of TTE, including defining the distal coronary course, proximal narrowing, and intramural course, thus providing critical information on the presence and depth of high-risk features [55,56]. The primary drawback to routine utilization of CCTA is the need for contrast administration and radiation exposure, though recent advances have reduced radiation doses comparable to a series of conventional chest radiographs [57].

Magnetic Resonance Imaging

Cardiac magnetic resonance (CMR) imaging also provides comprehensive coronary artery imaging and carries a class I indication for imaging AAOCA [23]. Like CCTA, CMR has the potential to define the entire coronary course and identify high-risk features [58]. Beyond TTE and CCTA, CMR can provide cardiac functional parameters and assess for regions of fibrosis [59]. CMR eliminates the risks of radiation and may be preferred over CCTA in younger athletes [60]. However, CMR is significantly more expensive and widespread use is limited by longer scan times and a lack of centers with appropriate technical expertise [6,58].

Invasive Coronary Imaging

With the inherent procedural risks and radiation exposure, invasive angiography is not considered first line in the initial evaluation of AAOCA [6,61]. However, IVUS can provide exceptional detail on stenosis severity and length of intramural courses [42]. IVUS may therefore represent an alternative option for risk stratification, particularly in cases in which CCTA and CMR lack sufficient detail [23,41]. The technical expertise required for IVUS, in combination with the potential procedural risks [62], limit the routine use of this modality as part of AAOCA evaluation [46].

AAOCA Surgical Correction Considerations

The ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease were recently updated in 2019, offering guidance to the surgical approach of patients with AAOCA [23]. The most recent athlete-specific guidelines for AAOCA were published in 2015 with the ACC/AHA Eligibility and Disqualification Recommendations for Athletes with Cardiovascular Abnormalities document [54]. All athletes with AAOCA and signs or symptoms suggestive of ischemia should be referred for corrective surgery (Class I recommendation) [23,54]. While the decision to proceed with surgery in the symptomatic, ischemic athlete with AAOCA is relatively straightforward, AAOCA is more commonly discovered in asymptomatic athletes. We will discuss these clinical situations separately for ALCA and ARCA.

ALCA

ALCA has been established as the most malignant AAOCA anatomic variation [14–16,21,22]. In Basso’s analysis of 27 athletes with SCD due to AAOCA, 23 had ALCA compared to just 4 with ARCA [2]. Similarly, in Eckart’s autopsy study of military personnel, each of the 21 AAOCA cases were identified as ALCA [20]. Maron also observed this pattern in American athletes with SCD, in which there were 65 cases of ALCA compared to 16 with ARCA [8]. At present, it is commonly accepted that because of the increased risk of SCD, all athletes with an interarterial ALCA should be referred for surgical repair before resuming sport participation [54].

ARCA

The presence of ARCA requires a more complex risk stratification and clinical decision-making algorithm. Following ACC/AHA guidelines, asymptomatic patients require ischemic risk stratification with stress testing and referral for surgical evaluation if positive (Class I recommendation) [23,54]. In consideration of risk of SCD, the need for surgical correction is uncertain with negative stress testing, particularly if high-risk phenotypic features are present. While the association between SCD and ALCA is more evident, data may be less compelling for ARCA and SCD. In a seminal postmortem study from Chietlin and colleagues, of the 51 subjects found to have AAOCA on autopsy, 18 were ARCA. Importantly, none of these individuals with ARCA died suddenly and 7 died from coronary artery disease [22]. The lower incidence of SCD in cases of ARCA has been replicated in multiple, subsequent pathologic studies [15,21,63]. Based on data from Maron [8], Brothers and colleagues estimated that the risk of sudden death over a 20-year period from ARCA may be as low as 0.2%, compared to 6.3% from ALCA [64].

The lack of demonstrable risk associated with ARCA has impacted recommendations for both surgical correction [23] and sport participation [54]. In contrast to prior recommendations [65], current ACC/AHA guidelines advocate that in cases of ARCA without signs or symptoms of ischemia, a return to sport can be considered with ongoing counseling and appropriate clinical follow up [54]. When counseling athletes with ARCA, it is imperative that all parties and stakeholders understand that accurate risk assessment of SCD due to ARCA remains uncertain.

Surgical Techniques

Surgical correction of AAOCA is considered safe with minimal perioperative mortality reported with current techniques [66,67]. Unroofing of the intramural segment is the most common technique performed in the United States [31,68]. Coronary translocation, in which the AAOCA is re-implanted into the correct sinus of Valsava, is sometimes preferred in those without significant intramural segments. Ostioplasty and side-to-side anastomoses are infrequently performed compared to unroofing or translocation procedures [69]. Coronary artery bypass grafting is generally avoided due to concerns of competitive flow and graft failure [23,70]. Despite advancements in surgical technique, large long-term prospective outcomes data are needed to determine survival improvement with surgery.

AAOCA Exercise Restrictions and Return to Play

With identification of AAOCA, either incidentally or due to symptoms, athletes should initially be restricted from competitive sport participation (with the possible exception of class IA activities such as bowling and golf) until appropriate risk stratification is completed [54]. For ALCA with an interarterial course, competitive sport restriction until surgical repair is recommended whether symptoms are present or not. For ARCA, further imaging studies, exercise stress testing, and functional assessments are recommended to determine if surgery is indicated [23]. Following surgical correction, athletes should be restricted from competitive sports for 3 months. After this period, athletes should be re-risk stratified with an exercise stress test to evaluate for symptoms or evidence of ischemia. Athletes may return to competitive exercise training if no inducible ischemia is present after maximal exercise stress testing and there are no ischemic symptoms present [54]. In cases where surgical correction is recommended but declined, the athlete should be restricted from all competitive sports with the exception of class IA activities [54].

Shared Decision-Making

With challenging cases of asymptomatic ARCA, uncertainties regarding risk of SCD in combination with ascertainment of potential high-risk imaging features may lead to complex management decisions. With limited data and inability to precisely quantify risk during intense exercise, determination of sports eligibility presents challenges for this population. At present, medical decision-making has shifted away from a paternalistic model. Rather, each case involves more individualized patient input, inclusive of personal motivation and values while also incorporating scientific evidence and guidelines when available. This paradigm shift to a shared decision-making model [10] is relevant to athletes and some cases of AAOCA when the precise risk of SCD may be unknown. Some athletes may prefer retirement rather than accept the unknown risks of SCD or surgery, while others may accept any level of risk in order to return to competition. As part of complicated sports eligibility decisions, the views of other stakeholders, such as a young athlete’s parents or sponsoring institutions must also be taken into account and incorporated in the shared decision-making process [11].

AAOCA Clinical Approach

We advocate for incorporation of standard ACC/AHA athlete guidelines [54] and implementation of protocols, specifically standardized TTE protocols, as part of the cadre of imaging studies obtained in a robust sports cardiology practice (Figure 2). While acknowledging the limitations inherent with coronary ostia visualization by TTE [24,51], experienced cardiac sonographers with prior pediatric or adult congenital backgrounds provide a high level of technical expertise necessary for achieving the appropriate sensitivity in identification of normal arising coronary ostia. TTE protocols should mandate using color Doppler flow during diastole to confirm the correct identification of the coronary ostia [50]. Further, specifically for the RCA, identifying the vessel in 2 views, parasternal and short axis, provides further evidence of the presence of a normal arising RCA when documenting color flow may be challenging [52,71]. With athletes presenting with concerning exertional symptoms suggestive of AAOCA (e.g. chest tightness), even with normal arising coronary ostia visualized by TTE, there should remain a low threshold to proceed with CCTA or CMR. Advanced imaging allows for better visualization of the entire course of the vessels and thus assessment for other congenital coronary anomalies. The initial approach for the symptomatic athlete should also include a resting ECG and maximal effort exercise testing to volitional fatigue.

Fig. 2. Algorithm for risk-stratification and management of athletes with anomalous aortic origin of the coronary arteries.

AAOCA: anomalous aortic origin of the coronary artery; ALCA: anomalous left coronary artery; ARCA: anomalous right coronary artery; CT: computed tomography; ECG: electrocardiogram

The ACC/AHA athletic guidelines [54] differ from the general adult congenital guidelines [23] with regard to asymptomatic patients with ALCA. Athlete-specific guidelines recommend surgical correction given the high-risk of SCD associated with intense physical activity with this phenotype [54]. Cases of ARCA are more complex from a sports eligibility standpoint. Return to play for asymptomatic athletes with ARCA, no high-risk features, and no inducible ischemia, is reasonable with careful counseling and clinical follow-up [54]. In cases of ARCA with no inducible ischemia but high-risk features present on imaging, clinical decision-making and sports eligibility considerations are challenging and should incorporate careful, in-depth discussions reviewing potential risks and shared decision-making with the athlete and key stakeholders [23]. Ultimately, more research and outcomes data are necessary to determine the most appropriate management strategies for athletic patients. We look forward to outcomes data from newly established AAOCA registries that will be instrumental in prospectively determining the risk associated with AAOCA variants and best practice clinical management strategies [31,46].

Conclusion

While uncommon in the general population, the presence of AAOCA poses unique diagnostic and clinical management considerations in athletes. Because of the strong association between AAOCA, SCD, and physical activity, athletes require a heightened awareness from practitioners for the possibility of this underlying condition. Unfortunately, symptoms are frequently absent and standard cardiovascular screening methods (physical exam, 12-lead ECG) are insensitive in detecting AAOCA. However, among diagnosed athletes with AAOCA, contemporary cardiac imaging modalities allow for risk stratification of AAOCA to identify those who may be at higher risk of SCD. As we await critically needed athlete-specific AAOCA outcomes data, clinical management strategies and sports eligibility considerations a part of complex AAOCA cases require a careful approach inclusive of detailed shared risk and decision-making processes.

Case Vignette #1:

A 19-year-old male collegiate soccer player complains of chest tightness during training. The discomfort is noted during high-intensity training and relieved with rest. Maximal exercise stress testing reveals anterior ST-segment depressions and cardiac imaging reveals an anomalous left coronary artery origin arising from the right sinus of Valsava.

Case Vignette #2:

A 55-year-old female long-term marathon runner is referred from primary care after a coronary calcium scan revealed an anomalous right coronary artery origin arising from the left sinus of Valsava. She is asymptomatic.