Expert consensus guidelines: Anomalous aortic origin of a coronary artery

Abstract

Central MessageWe sought to establish evidence-based guidelines for the management anomalous aortic origin of a coronary artery.PerspectiveAnomalous aortic origin of a coronary artery is associated with sudden cardiac death. Although the risk for any single affected individual is small, the loss of an otherwise healthy person is particularly devastating. Surgical and interventional therapies have been developed that appear to be protective but these therapies carry risks. The challenge is identifying those individuals at such risk.See Editorial Commentary page 1458.See Editorial page 1462. We sought to establish evidence-based guidelines for the management anomalous aortic origin of a coronary artery. Anomalous aortic origin of a coronary artery is associated with sudden cardiac death. Although the risk for any single affected individual is small, the loss of an otherwise healthy person is particularly devastating. Surgical and interventional therapies have been developed that appear to be protective but these therapies carry risks. The challenge is identifying those individuals at such risk. See Editorial Commentary page 1458. See Editorial page 1462. The objective of this project was to establish consensus 2016 American Association for Thoracic Surgery (AATS) evidence-based guidelines for the management of anomalous aortic origin of a coronary artery. In many types of coronary anomalies, the risk of sudden cardiac death (SCD) is largely unknown, as the anomalies are quite rare. However, observational studies have identified the coronary anomalies that appear to be most prevalent and in which the SCD risk appears to be the greatest: when both coronary arteries arise from the same aortic sinus with either a single ostium or 2 separate ostia (Table 1). The aberrant vessel may arise above the inappropriate sinus or above the commissure and not truly from the sinus itself. For this article, we refer to this as anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate coronary sinus. The course the anomalous coronary artery takes appears to have an impact on its risk of SCD. The anomalous aortic origin of the left main coronary artery (AAOLCA) or right coronary artery (AAORCA) can course in front of the pulmonary artery (pre-pulmonic) or posterior to the aorta (posterior/retroaortic). The AAOLCA or left anterior descending coronary artery alone can course through the conal septum (intraseptal or intraconal or intramyocardial). These previous subtypes are generally not believed to be clinically significant. However, the course becomes important if either the aberrant left main coronary artery or right coronary artery travels between the 2 great vessels. This review and recommendations are directed at AAOCA from the opposite sinus of Valsalva with an interarterial course (Figure 1).Table 1Variations of anomalous aortic origin of a coronary arteryAdapted from Brothers JA, Gaynor JW. Coronary artery anomalies in children. In: Kaiser LR, Kron IL, Spray TL, eds. Mastery of Cardiothoracic Surgery. 3rd ed. Philadelphia, PA: Lippincott, William and Wilkins; 2014:1055-73.1.Left main coronary artery (LMCA) arises from right sinus of Valsalva, either directly from right coronary artery or as a separate ostiaa.LMCA course anterior to pulmonary artery (anterior)b.LMCA course posterior to aorta (posterior)c.LMCA course through interventricular septum (intraseptal/intraconal)d.LMCA course between aorta and pulmonary artery (interarterial)2.Left anterior descending coronary or left circumflex coronary artery may originate from the right sinus alone3.Right coronary artery (RCA) arises from left sinus of Valsalva, either from LMCA or as a separate ostiaa.RCA courses anterior to pulmonary artery (anterior)b.RCA courses posterior to aorta (posterior)c.RCA courses between aorta and pulmonary artery (interarterial)4.Single LMCA arises from left sinus of Valsalva and bifurcates into the left anterior descending coronary and left circumflex coronary arteries. The left circumflex coronary artery crosses the crux and continues as the RCA.5.Single RCA arises from right sinus, crosses crux, continues as left anterior descending coronary and left circumflex coronary arteries Open table in a new tab The AATS Guidelines Committee identified the management of AAOCA as a key topic in cardiothoracic surgery suitable for the establishment of clinical guidelines. The Guidelines Committee selected James S. Tweddell, MD, as chair of the AAOCA Working Group and asked him to appoint an AAOCA Guidelines writing committee to create evidence-based guidelines for the AATS Guidelines Committee. The chair assembled a multidisciplinary group of experts, the coauthors of this article, who include congenital cardiac surgeons and adult and pediatric cardiologists. Members were tasked with performing comprehensive literature searches, and making recommendations based on a review of the literature. Members also graded the quality of the evidence supporting the recommendations and with assessing the risk-benefit profile for each recommendation. The level of evidence was graded by the work force panel according to standards published by the Institute of Medicine (Figure 2). For the development of the guidelines, we followed the recommendations of the Institute of Medicine's 2011 Clinical Practice Guidelines We Can Trust: Standards for Developing Trustworthy Clinical Practice Guidelines (http://www.nationalacademies.org/hmd/Reports/2011/Clinical-Practice-Guidelines-We-Can-Trust.aspx). Scheduled teleconferences were used to organize the topics to be covered by the guidelines, review the literature review summaries, and propose recommendations. For all meetings, agendas were circulated beforehand. Summaries of the conference calls were circulated to the writing committee members. The final recommendations were voted on by the writing committee to present to the Councilors of the AATS and review the final manuscript. The writing committee unanimously agreed on all recommendations. Therefore, the process followed the recommendations of the Institute of Medicine, but an extensive consultation with all other stakeholders, including patients, was not performed. Instead, the AATS guidelines attempt to provide a rapid response to the rapidly changing medical literature and provide clinicians with the recommendations of senior content experts based on the best available information. The expert consensus provides important guidance to clinicians, particularly when the quality of the evidence is limited or contradictory. These consensus guidelines provide the best opinion of a group of content experts. The following recommendations are based on expert consensus opinion as well as on the best available evidence. Despite important knowledge gaps, we feel it is important and timely to review the available evidence and present expert opinion based on best practices. The guidelines were prepared for publication in The Journal of Thoracic and Cardiovascular Surgery. The true prevalence of congenital coronary anomalies that are potentially pathologic in the general population is difficult to ascertain. Several studies have attempted to quantify this value, with estimates ranging between 0.1% and 1.0% in both the adult and pediatric populations.1Yamanaka O. Hobbs R.E. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography.Cathet Cardiovasc Diagn. 1990; 21: 28-40Crossref PubMed Google Scholar, 2Tuo G. Marasini M. Brunelli C. Zannini L. Balbi M. Incidence and clinical relevance of primary congenital anomalies of the coronary arteries in children and adults.Cardiol Young. 2013; 23: 381-386Crossref PubMed Google Scholar, 3Labombarda F. Coutance G. Pellissier A. Mery-Alexandre C. 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Novel imaging of coronary artery anomalies to assess their prevalence, the causes of clinical symptoms, and the risk of sudden cardiac death.Circ Cardiovasc Imaging. 2014; 7: 747-754Crossref PubMed Scopus (0) Google Scholar The wide difference in prevalence rates is likely due to choice of imaging modality, patient population chosen to study, and/or the how coronary anomalies were defined.8Angelini P. Coronary artery anomalies: an entity in search of an identity.Circulation. 2007; 115: 1296-1305Crossref PubMed Scopus (0) Google Scholar When looking specifically at the estimated prevalence of interarterial AAOCA, this has ranged between 0.1% and 0.7%.1Yamanaka O. Hobbs R.E. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography.Cathet Cardiovasc Diagn. 1990; 21: 28-40Crossref PubMed Google Scholar, 2Tuo G. Marasini M. Brunelli C. Zannini L. Balbi M. Incidence and clinical relevance of primary congenital anomalies of the coronary arteries in children and adults.Cardiol Young. 2013; 23: 381-386Crossref PubMed Google Scholar, 3Labombarda F. Coutance G. Pellissier A. Mery-Alexandre C. Roule V. Maragnes P. et al.Major congenital coronary artery anomalies in a paediatric and adult population: a prospective echocardiographic study.Eur Heart J Cardiovasc Imaging. 2014; 15: 761-768Crossref PubMed Scopus (0) Google Scholar, 4Pelliccia A. Spataro A. Maron B.J. Prospective echocardiographic screening for coronary artery anomalies in 1,360 elite competitive athletes.Am J Cardiol. 1993; 72: 978-979Abstract Full Text PDF PubMed Scopus (91) Google Scholar, 5Davis J.A. Cecchin F. Jones T.K. Portman M.A. Major coronary artery anomalies in a pediatric population: incidence and clinical importance.J Am Coll Cardiol. 2001; 37: 593-597Crossref PubMed Scopus (230) Google Scholar, 6Maron B.J. Shirani J. Poliac L.C. Mathenge R. Roberts W.C. Mueller F.O. Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles.JAMA. 1996; 276: 199-204Crossref PubMed Google Scholar, 7Angelini P. Novel imaging of coronary artery anomalies to assess their prevalence, the causes of clinical symptoms, and the risk of sudden cardiac death.Circ Cardiovasc Imaging. 2014; 7: 747-754Crossref PubMed Scopus (0) Google Scholar Yamanaka and Hobbs1Yamanaka O. Hobbs R.E. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography.Cathet Cardiovasc Diagn. 1990; 21: 28-40Crossref PubMed Google Scholar found the incidence to be approximately 0.3% of more than 100,000 adults evaluated with coronary angiography. In a large population-based prospective study in asymptomatic children using transthoracic echocardiography, the incidence of AAOCA was found to be 0.17%.5Davis J.A. Cecchin F. Jones T.K. Portman M.A. Major coronary artery anomalies in a pediatric population: incidence and clinical importance.J Am Coll Cardiol. 2001; 37: 593-597Crossref PubMed Scopus (230) Google Scholar Because this study evaluated only asymptomatic patients, the true prevalence was likely underestimated, as they did not include those children with symptomatic AAOCA. Recently, Angelini7Angelini P. Novel imaging of coronary artery anomalies to assess their prevalence, the causes of clinical symptoms, and the risk of sudden cardiac death.Circ Cardiovasc Imaging. 2014; 7: 747-754Crossref PubMed Scopus (0) Google Scholar reported on a magnetic resonance imaging screening program designed to identify high-risk cardiac lesions, and found 0.7% of the population screened had interarterial AAOCA, with 0.5% interarterial AAORCA. If we extrapolate this to the population of young people (ages 12-35) in the United States, this could mean more than 600,000 children and young adults have interarterial AAOCA. In most studies, interarterial AAORCA is from 3 to 6 times more common than AAOLCA.1Yamanaka O. Hobbs R.E. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography.Cathet Cardiovasc Diagn. 1990; 21: 28-40Crossref PubMed Google Scholar, 7Angelini P. Novel imaging of coronary artery anomalies to assess their prevalence, the causes of clinical symptoms, and the risk of sudden cardiac death.Circ Cardiovasc Imaging. 2014; 7: 747-754Crossref PubMed Scopus (0) Google Scholar Little is known about the genetics of coronary anomalies, especially those that have the potential for sudden death, including interarterial AAOCA. The development of the coronary arteries is complex, with many different sites at which a mutation leads to a potentially lethal coronary anomaly. There are minimal data regarding the genetic patterns of AAOCA. Bloor et al9Bloor C.M. Leon A.S. Pitt B. The inheritance of coronary artery anatomic patterns in rats.Circulation. 1967; 36: 771-776Crossref PubMed Google Scholar reported on the coronary artery anatomy in albino rats and found that the genetic variation in newborn rats and their parents was likely determined from multiple genes and not of classical Mendelian inheritance. There are no published data about the genetic inheritance in humans. However, there have been reports of families with at least 2 first-degree relatives affected by AAOCA.10Laureti J.M. Singh K. Blankenship J. Anomalous coronary arteries: a familial clustering.Clin Cardiol. 2005; 28: 488-490Crossref PubMed Scopus (0) Google Scholar, 11Brothers J.A. Stephens P. Gaynor J.W. Lorber R. Vricella L.A. Paridon S.M. Anomalous aortic origin of a coronary artery with an interarterial course: should family screening be routine?.J Am Coll Cardiol. 2008; 51: 2062-2064Crossref PubMed Scopus (0) Google Scholar, 12Devanagondi R. Brenner J. Vricella L. Ravekes W. A tale of two brothers: anomalous coronary arteries in two siblings.Pediatr Cardiol. 2008; 29: 816-819Crossref PubMed Scopus (0) Google Scholar Genetic testing in association with focused imaging of the relatives of the AAOCA patient may help elucidate any potential genetic factors. In a more general, nonspecific sense, there are multiple population studies demonstrating familial clustering of sudden cardiac arrest (SCA) as a first clinical manifestation of ischemia.13Friedlander Y. Siscovick D.S. Weinmann S. Austin M.A. Psaty B.M. Lemaitre R.N. et al.Family history as a risk factor for primary cardiac arrest.Circulation. 1998; 97: 155-160Crossref PubMed Google Scholar, 14Jouven X. Desnos M. Guerot C. Ducimetiere P. Predicting sudden death in the population: the Paris Prospective Study I.Circulation. 1999; 99: 1978-1983Crossref PubMed Google Scholar, 15Dekker L.R. Bezzina C.R. Henriques J.P. Tanck M.W. Koch K.T. Alings M.W. et al.Familial sudden death is an important risk factor for primary ventricular fibrillation: a case-control study in acute myocardial infarction patients.Circulation. 2006; 114: 1140-1145Crossref PubMed Scopus (179) Google Scholar, 16Kaikkonen K.S. Kortelainen M.L. Linna E. Huikuri H.V. Family history and the risk of sudden cardiac death as a manifestation of an acute coronary event.Circulation. 2006; 114: 1462-1467Crossref PubMed Scopus (110) Google Scholar This suggests a yet-undefined genetic basis for an arrhythmic response to ischemia, distinct from, but in addition to, any genetic basis for the primary structural defect itself. Since the exact mechanism of SCD associated with coronary anomalies is not known, several hypotheses have been put forth based on anatomic and physiologic properties of the anomalous coronary. Certain factors appear to predispose to myocardial ischemia and/or lethal ventricular arrhythmias, likely due to limitation of coronary reserve. These include 1 or more of the following: ostial stenosis in association with an oblique take-off from the aorta, ostial ridge, vessel spasm, intussusception, noncompliant pericommissural area, and compression of the anomalous coronary artery intramurally and/or between the great arteries.17Cheitlin M.D. De Castro C.M. McAllister H.A. Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva, a not-so-minor congenital anomaly.Circulation. 1974; 50: 780-787Crossref PubMed Google Scholar, 18Taylor A.J. Byers J.P. Cheitlin M.D. Virmani R. Anomalous right or left coronary artery from the contralateral coronary sinus: “high-risk” abnormalities in the initial coronary artery course and heterogeneous clinical outcomes.Am Heart J. 1997; 133: 428-435Abstract Full Text Full Text PDF PubMed Google Scholar, 19Roberts W.C. Siegel R.J. Zipes D.P. Origin of the right coronary artery from the left sinus of valsalva and its functional consequences: analysis of 10 necropsy patients.Am J Cardiol. 1982; 49: 863-868Abstract Full Text PDF PubMed Scopus (240) Google Scholar It remains unknown whether these mechanisms act alone or in combination to provide the substrate for SCD. The most common anomaly that appears to carry some increased risk of SCD is AAORCA. However, interarterial AAOLCA is proportionally far more prevalent among individuals who have died suddenly with no other explanation.17Cheitlin M.D. De Castro C.M. McAllister H.A. Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva, a not-so-minor congenital anomaly.Circulation. 1974; 50: 780-787Crossref PubMed Google Scholar, 18Taylor A.J. Byers J.P. Cheitlin M.D. Virmani R. Anomalous right or left coronary artery from the contralateral coronary sinus: “high-risk” abnormalities in the initial coronary artery course and heterogeneous clinical outcomes.Am Heart J. 1997; 133: 428-435Abstract Full Text Full Text PDF PubMed Google Scholar, 19Roberts W.C. Siegel R.J. Zipes D.P. Origin of the right coronary artery from the left sinus of valsalva and its functional consequences: analysis of 10 necropsy patients.Am J Cardiol. 1982; 49: 863-868Abstract Full Text PDF PubMed Scopus (240) Google Scholar, 20Taylor A.J. Rogan K.M. Virmani R. Sudden cardiac death associated with isolated congenital coronary artery anomalies.J Am Coll Cardiol. 1992; 20: 640-647Crossref PubMed Scopus (573) Google Scholar, 21Kragel A.H. Roberts W.C. Anomalous origin of either the right or left main coronary artery from the aorta with subsequent coursing between aorta and pulmonary trunk: analysis of 32 necropsy cases.Am J Cardiol. 1988; 62: 771-777Abstract Full Text PDF PubMed Scopus (230) Google Scholar Despite not knowing the exact mechanism of ischemia in those with AAOCA, identifying and treating those anomalies that place the patient at risk of SCD is of utmost importance, and management should ideally be based on the risk assessment. Although the true risk is unknown, an estimation based on some assumptions can be calculated. The mortality rates frequently cited and used for risk assessment are from autopsy series data and include 0% to 57% for AAORCA and 27% to 100% for AAOLCA.17Cheitlin M.D. De Castro C.M. McAllister H.A. Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva, a not-so-minor congenital anomaly.Circulation. 1974; 50: 780-787Crossref PubMed Google Scholar, 18Taylor A.J. Byers J.P. Cheitlin M.D. Virmani R. Anomalous right or left coronary artery from the contralateral coronary sinus: “high-risk” abnormalities in the initial coronary artery course and heterogeneous clinical outcomes.Am Heart J. 1997; 133: 428-435Abstract Full Text Full Text PDF PubMed Google Scholar, 19Roberts W.C. Siegel R.J. Zipes D.P. Origin of the right coronary artery from the left sinus of valsalva and its functional consequences: analysis of 10 necropsy patients.Am J Cardiol. 1982; 49: 863-868Abstract Full Text PDF PubMed Scopus (240) Google Scholar, 20Taylor A.J. Rogan K.M. Virmani R. Sudden cardiac death associated with isolated congenital coronary artery anomalies.J Am Coll Cardiol. 1992; 20: 640-647Crossref PubMed Scopus (573) Google Scholar, 21Kragel A.H. Roberts W.C. Anomalous origin of either the right or left main coronary artery from the aorta with subsequent coursing between aorta and pulmonary trunk: analysis of 32 necropsy cases.Am J Cardiol. 1988; 62: 771-777Abstract Full Text PDF PubMed Scopus (230) Google Scholar What is necessary to understand is that these rates reflect the prevalence of AAOCA in those who have already died and not the risk of SCD in those living with AAOCA. Recently, 2 studies reported on the incidence of SCD or SCA in people 35 years and younger.22Meyer L. Stubbs B. Fahrenbruch C. Maeda C. Harmon K. Eisenberg M. et al.Incidence, causes, and survival trends from cardiovascular-related sudden cardiac arrest in children and young adults 0 to 35 years of age: a 30-year review.Circulation. 2012; 126: 1363-1372Crossref PubMed Scopus (130) Google Scholar, 23Pilmer C.M. Kirsh J.A. Hildebrandt D. Krahn A.D. Gow R.M. Sudden cardiac death in children and adolescents between 1 and 19 years of age.Heart Rhythm. 2014; 11: 239-245Abstract Full Text Full Text PDF PubMed Scopus (45) Google Scholar In the combined cohorts, there were 4 cases of AAOCA associated with SCD or SCA in a combined 34 million patient-years; in 1 study, the 2 cases of SCD (ages <19 years) were both interarterial AAOLCA and known to be associated with vigorous physical activity.23Pilmer C.M. Kirsh J.A. Hildebrandt D. Krahn A.D. Gow R.M. Sudden cardiac death in children and adolescents between 1 and 19 years of age.Heart Rhythm. 2014; 11: 239-245Abstract Full Text Full Text PDF PubMed Scopus (45) Google Scholar These studies demonstrate that the risk of SCD or SCA in the young, in the absence of participation in competitive sports, is exceedingly low.24Basso C. Maron B.J. Corrado D. Thiene G. Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes.J Am Coll Cardiol. 2000; 35: 1493-1501Crossref PubMed Scopus (696) Google Scholar Vigorous physical activity increases the risk of SCD in those with interarterial AAOCA.6Maron B.J. Shirani J. Poliac L.C. Mathenge R. Roberts W.C. Mueller F.O. Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles.JAMA. 1996; 276: 199-204Crossref PubMed Google Scholar, 23Pilmer C.M. Kirsh J.A. Hildebrandt D. Krahn A.D. Gow R.M. Sudden cardiac death in children and adolescents between 1 and 19 years of age.Heart Rhythm. 2014; 11: 239-245Abstract Full Text Full Text PDF PubMed Scopus (45) Google Scholar, 25Maron B.J. Doerer J.J. Haas T.S. Tierney D.M. Mueller F.O. Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980-2006.Circulation. 2009; 119: 1085-1092Crossref PubMed Scopus (882) Google Scholar Maron et al25Maron B.J. Doerer J.J. Haas T.S. Tierney D.M. Mueller F.O. Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980-2006.Circulation. 2009; 119: 1085-1092Crossref PubMed Scopus (882) Google Scholar published a comprehensive analysis of sudden deaths among competitive athletes in the United States over a 27-year period. Of the 690 SCD episodes ascribed to a cardiovascular cause, 119 of these were due to AAOCA. Based on their data, the incidence of SCD would be approximately 0.1 per 100,000 person-years from AAOCA. A similar incidence of SCD was found among Minnesota high school athletes over a 26-year period, with 2 SCD events occurring due to AAOCA, both of which were AAOLCA.26Maron B.J. Haas T.S. Ahluwalia A. Rutten-Ramos S.C. Incidence of cardiovascular sudden deaths in Minnesota high school athletes.Heart Rhythm. 2013; 10: 374-377Abstract Full Text Full Text PDF PubMed Scopus (59) Google Scholar Using the data of Maron et al,25Maron B.J. Doerer J.J. Haas T.S. Tierney D.M. Mueller F.O. Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980-2006.Circulation. 2009; 119: 1085-1092Crossref PubMed Scopus (882) Google Scholar Brothers et al27Brothers J. Carter C. McBride M. Spray T. Paridon S. Anomalous left coronary artery origin from the opposite sinus of Valsalva: evidence of intermittent ischemia.J Thorac Cardiovasc Surg. 2010; 140: e27-e29Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar calculated the cumulative risk of death over a 20-year period in children and young adults with AAOCA (ages 15-35 years) participating in competitive sports was 6.3% for AAOLCA and 0.2% for AAORCA. Even though these analyses are prone to ascertainment bias as well as underreporting, it does seem that the risk of SCD ascribed to AAOCA is far less than reported in autopsy series. It is important to understand that most risk estimates are based on the presumed incidence of SCD in those who are participating in competitive athletics, which is only approximately 10% to 15% of children and adolescents. They do not assess risk among those who have SCA during high-intensity recreational sports, or in the general population not participating in higher-level sports. Competitive athletes are defined as individuals of middle school age and older (generally ≥11 years of age) who are engaged in exercise training on a regular basis and participate in official sports competition organized by a recognized athletic association. Competitive athletes place a high premium on athletic excellence and these individuals typically exercise more than 10 hours per week. In contrast, recreational athletes are defined as individuals engaged in recreational or leisure-time sports activities, on either a regular basis or intermittently. Usually, they exercise less than 10 hours per week. Recreational sports do not necessarily require systematic training or the pursuit of excellence.28Solberg E.E. Borjesson M. Sharma S. Papadakis M. Wilhelm M. Drezner J.A. et al.Sudden cardiac arrest in sports—need for uniform registration: a position paper from the Sport Cardiology Section of the European Association for Cardiovascular Prevention and Rehabilitation.Eur J Prev Cardiol. 2016; 23: 657-667Crossref PubMed Google Scholar Although there are rare case reports of SCD from AAOCA at rest or while participating in recreational activity, these reports are just that: individual cases that occur rarely, and not derived from any formal databases. Notably, a recent study from France demonstrated that many more SCDs occur during recreational sports than during competitive activities, albeit at an older age.29Marijon E. Tafflet M. Celermajer D.S. Dumas F. Perier M.C. Mustafic H. et al.Sports-related sudden death in the general population.Circulation. 2011; 124: 672-681Crossref PubMed Scopus (203) Google Scholar This increasing number of older individuals (eg, ages late 20s to 40s) with reports of SCD or SCA from AAOCA may be due to the growth of sanctioned sporting events for adults, such as triathlons and running and bicycle races. In fact, these older athletes may now be exerting themselves at a higher level than they did when they were young. Taking a conservative prevalence rate of AAOCA in the population of approximately 0.2%, which may be underestimating the prevalence based on recent studies, then there are at least 600,000 young people in the United States with AAOCA. Besides the competitive athlete, in whom there are only a small number of SCD events reported every year, the risk of SCD for the asymptomatic young person with interarterial AAOCA, even AAOLCA, who is not participating in competitive sports does not appear to be significantly greater than the SCD risk for those without AAOCA who are participating only in recreational athletics. There is not a typical way that patients present with AAOCA. For some, the initial presentation is aborted SCD or true SCD. However, most patients with AAOCA are diagnosed when the anomaly is found incidentally on a transthoracic or transesophageal echocardiogram or computed tomography (CT) angiogram that is performed for another reason, such as a heart murmur or abnormal electrocardiogram (ECG). The patient may also have an echocardiogram performed for symptoms related to exertion, such as chest pain, palpitations, dizziness, presyncope, or syncope. Although many of these symptoms are often seen in those without coronary anomalies, these complaints prompt the referral to a cardiologist and an echocardiogram is performed. Chest pain should be considered ischemic if it is accompanied by evidence of myocardial injury, noted by ST segment depression at rest or with exercise, ventricular arrhythmias that increase with exercise, lack of increase or a decrease in blood pressure with exercise, evidence of wall motion abnormality on echocardiography, perfusion defect on nuclear scan in the correct distribution of the anomalous coronary artery, and/or evidence of past fibrosis/scar or perfusion abnormality seen by cardiac magnetic resonance imaging (MRI). Recent large-scale screening programs have increased the frequency of incidentally diagnosed AAOCA. A screening ECG is not reliable for suspecting or recognizing AAOCA.30Roberts W.O. Asplund C.A. O'Connor F.G. Stovitz S.D. Cardiac preparticipation screening for the young athlete: why the routine use of ECG is not necessary.J Electrocardiol. 2015; 48: 311-315Abstract Full Text Full Text PDF PubMed Google Scholar The presence of a q-wave consisten