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We sought to establish evidence-based guidelines for the management anomalous aortic origin of a coronary artery. Perspective 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. Rationale and ObjectivesThe 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 artery Adapted 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.
 Figure 1Transthoracic echocardiography from a short-axis plane in a patient with anomalous origin of the right coronary artery from the left sinus of Valsalva and an intramural course of the anomalous coronary. A, The anomalous right coronary artery can be seen arising from the left sinus of Valsalva near the origin of the left main coronary artery (LMCA) and coursing intramurally within the anterior aortic wall (arrowheads) between the aorta (Ao) and the pulmonary artery (PA) toward the right sinus of Valsalva. B, Color Doppler imaging shows the linear diastolic flow of the anomalous coronary within the anterior aortic wall (arrow); the red color signal confirms anomalous coronary flow towards the transducer anteriorly, consistent with the coronary originating from the left sinus and coursing toward the more anteriorly positioned right sinus. Reprinted with permission. 53
Prospective echocardiographic diagnosis and surgical repair of anomalous origin of a coronary artery from the opposite sinus with an interarterial course.
Methods of ReviewThe 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. Section I: BackgroundPrevalenceThe 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. , 2
Incidence and clinical relevance of primary congenital anomalies of the coronary arteries in children and adults.
, 3
Major congenital coronary artery anomalies in a paediatric and adult population: a prospective echocardiographic study.
, , , 6
Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles.
, 7
Novel imaging of coronary artery anomalies to assess their prevalence, the causes of clinical symptoms, and the risk of sudden cardiac death.
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. When looking specifically at the estimated prevalence of interarterial AAOCA, this has ranged between 0.1% and 0.7%. , 2
Incidence and clinical relevance of primary congenital anomalies of the coronary arteries in children and adults.
, 3
Major congenital coronary artery anomalies in a paediatric and adult population: a prospective echocardiographic study.
, , , 6
Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles.
, 7
Novel imaging of coronary artery anomalies to assess their prevalence, the causes of clinical symptoms, and the risk of sudden cardiac death.
Yamanaka and Hobbs 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%. Because this study evaluated only asymptomatic patients, the true prevalence was likely underestimated, as they did not include those children with symptomatic AAOCA. Recently, Angelini 7
Novel imaging of coronary artery anomalies to assess their prevalence, the causes of clinical symptoms, and the risk of sudden cardiac death.
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. , 7
Novel imaging of coronary artery anomalies to assess their prevalence, the causes of clinical symptoms, and the risk of sudden cardiac death.
GeneticsLittle 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 al 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. , 11
Anomalous aortic origin of a coronary artery with an interarterial course: should family screening be routine?.
, 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. 13
Family history as a risk factor for primary cardiac arrest.
, 14
Predicting sudden death in the population: the Paris Prospective Study I.
, 15
Familial sudden death is an important risk factor for primary ventricular fibrillation: a case-control study in acute myocardial infarction patients.
, 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. SCD and the Mechanism of IschemiaSince 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. 17
Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva, a not-so-minor congenital anomaly.
, 18
Anomalous right or left coronary artery from the contralateral coronary sinus: “high-risk” abnormalities in the initial coronary artery course and heterogeneous clinical outcomes.
, It remains unknown whether these mechanisms act alone or in combination to provide the substrate for SCD. Risk of SCDThe 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. 17
Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva, a not-so-minor congenital anomaly.
, 18
Anomalous right or left coronary artery from the contralateral coronary sinus: “high-risk” abnormalities in the initial coronary artery course and heterogeneous clinical outcomes.
, , , 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. 17
Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva, a not-so-minor congenital anomaly.
, 18
Anomalous right or left coronary artery from the contralateral coronary sinus: “high-risk” abnormalities in the initial coronary artery course and heterogeneous clinical outcomes.
, , , 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. 22
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.
, 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. These studies demonstrate that the risk of SCD or SCA in the young, in the absence of participation in competitive sports, is exceedingly low. 24
Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes.
Exercise and SCD riskVigorous physical activity increases the risk of SCD in those with interarterial AAOCA. 6
Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles.
, , Maron et al 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. Using the data of Maron et al, Brothers et al 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. 28
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.
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. 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. Section II: Presentation and DiagnosisPresentationThere 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. Diagnostic StudiesA screening ECG is not reliable for suspecting or recognizing AAOCA. The presence of a q-wave consistent with a previous myocardial infarction scar is rarely, if ever, seen in AAOCA. Exertional chest pain or dyspnea in a relatively young individual not otherwise suspected to be at risk for coronary atherosclerosis may be helpful; however, 2 reports suggest that 50% of SCD associated with coronary artery anomalies were first events without previous symptoms. 24
Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes.
, 31
Sudden death in young adults: a 25-year review of autopsies in military recruits.
As well, stress tests are not uniformly positive among individuals with these anomalies. 24
Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes.
, 32
Anomalous origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course: clinical profile and approach to management in the pediatric population.
In the past, cardiac catheterization with coronary angiography was routinely used for diagnosis of AAOCA; however, due to the invasive nature of the test and its inherent exposure to ionizing radiation, it is rarely used in the pediatric population. Cardiac catheterization is indicated if the anatomy cannot be defined with noninvasive imaging and in adults with suspected or echocardiographically defined anomalous vessels, for both complete definition of the anatomy and concomitant evaluation for coronary atherosclerotic disease. , 33
A retrospective study of angiographic ally determined anomalous coronary arteries in 12,844 subjects in Thrace region of Turkey.
, 34
Coronary arterial anomalies in a large group of patients undergoing coronary angiography in southeast Turkey.
, , 36
Anomalous origin of the right coronary artery from the left coronary sinus is associated with early development of coronary artery disease.
, , 38
Angiographic identification of primary coronary anomalies causing impaired myocardial perfusion.
, 39
Coronary intervention in anomalous origin of the right coronary artery (ARCA) from the left sinus of valsalva (LSOV): a single center experience.
, , 41
Three different situations and approaches in the management for anomalous origin of the right coronary artery from the left coronary sinus: case report.
, 42
The primary anomalies of coronary artery origin and course: a coronary angiographic analysis of 16,573 patients.
, 43
Prevalence of coronary artery anomalies in 12,457 adult patients who underwent coronary angiography.
, 44
Congenital anomalous aortic origins of the coronary arteries in adults: a Tunisian coronary arteriography study.
, 45
Anomalous origin of right coronary artery from left coronary sinus.
, 46
The anomalous origin of the left coronary artery from the right aortic sinus: is the coronary angiography still a ‘gold standard'?.
, 47
Frequency in the anomalous origin of the left main coronary artery with angiography in a Turkish population.
, , , 50
Primary angioplasty in acute inferior myocardial infarction with anomalous-origin right coronary arteries as infarct-related arteries: focus on anatomic and clinical features, outcomes, selection of guiding catheters and management.
, , 52
Magnetic resonance angiography of anomalous coronary arteries. A new gold standard for delineating the proximal course?.
Obviously, it is not used for routine screening in the absence of ischemic symptoms or clues from other imaging studies. The best method for initially identifying AAOCA is a carefully performed transthoracic echocardiogram with Doppler color flow mapping. 11
Anomalous aortic origin of a coronary artery with an interarterial course: should family screening be routine?.
, , 32
Anomalous origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course: clinical profile and approach to management in the pediatric population.
, 53
Prospective echocardiographic diagnosis and surgical repair of anomalous origin of a coronary artery from the opposite sinus with an interarterial course.
, 54
Utility and importance of new echocardiographic screening methods in diagnosis of anomalous coronary origins in the pediatric population: assessment of quality improvement.
, 55
Anomalous left main coronary artery origin from the right sinus of Valsalva: a novel echocardiographic screening method.
, 56
Anomalous aortic origin of a coronary artery: preoperative diagnosis and surgical planning.
, , , , , 61
Aborted sudden death in a young football player due to anomalous origin of the left coronary artery: successful surgical correction.
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A comparison of perioperative management of anomalous aortic origin of a coronary artery between an adult and pediatric cardiac center.
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63
Utility of multimodality imaging in the morphologic characterization of anomalous aortic origin of a coronary artery.
, 64
Anomalous aortic origin of coronary arteries in the young: echocardiographic evaluation with surgical correlation.
, This is generally the initial diagnostic modality due to availability, cost-effectiveness, ease of performance, and absence of radiation exposure. Imaging should clarify origin of left or right, as well as the presence or absence of an intramural course. Additional imaging is indicated when the anatomy cannot be accurately defined. 64
Anomalous aortic origin of coronary arteries in the young: echocardiographic evaluation with surgical correlation.
Coronary CT angiography or cardiac MRI is commonly used to obtain better visualization of the coronary artery anatomy to confirm the diagnosis. , 46
The anomalous origin of the left coronary artery from the right aortic sinus: is the coronary angiography still a ‘gold standard'?.
, 52
Magnetic resonance angiography of anomalous coronary arteries. A new gold standard for delineating the proximal course?.
, , 63
Utility of multimodality imaging in the morphologic characterization of anomalous aortic origin of a coronary artery.
, , , , 69
Anomalous coronary arteries in adults: depiction at multi-detector row CT angiography.
, , 71
A review of anomalous origination of a coronary artery from an opposite sinus of Valsalva (ACAOS) impact on major adverse cardiovascular events based on coronary computerized tomography angiography: a 6-year single center review.
, , , 74
Prevalence and characteristics of coronary anomalies originating from the opposite sinus of Valsalva in 8,522 patients referred for coronary computed tomography angiography.
, 75
Prevalence of congenital coronary artery anomalies of Korean men detected by coronary computed tomography.
, , , , 79
Computed tomographic angiography identification of intramural segments in anomalous coronary arteries with interarterial course.
, , , , 83
Anomalous origin of the right coronary artery originating from the left coronary sinus of Valsalva with an interarterial course: diagnosis and dynamic evaluation using dual-source computed tomography.
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Congenital coronary artery anomalies: diagnosis with 64 slice multidetector row computed tomography coronary angiography: a single-centre study.
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Multislice computed tomographic findings of the anomalous origins of the right coronary artery: evaluation of possible causes of myocardial ischemia.
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Anomalous origin of the three coronary arteries from the right aortic sinus Valsalva: role of MDCT coronary angiography.
, 87
Congenital anomalies of the coronary arteries: imaging with contrast-enhanced, multidetector computed tomography.
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Detection of anomalous origins of the coronary artery by means of multislice computed tomography.
, 89
Cardiac magnetic resonance imaging characterizes stenosis, perfusion, and fibrosis preoperatively and postoperatively in children with anomalous coronary arteries.
, 90
Cardiac MRI and CT: differentiation of normal ostium and intraseptal course from slitlike ostium and interarterial course in anomalous left coronary artery in children.
, , 92
Screening for proximal coronary artery anomalies with 3-dimensional MR coronary angiography.
, , 94
Depiction of anomalous coronary vessels and their relation to the great arteries by magnetic resonance angiography.
, Once the anatomy has been established, a maximal exercise stress test should be used to help assess the potential ischemic burden of the anatomic variant, especially in competitive athletes or high-intensity recreational athletes. We also recommend that the exercise test be combined with a nuclear perfusion scan or stress echocardiogram to optimize the sensitivity of identifying ischemia , 32
Anomalous origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course: clinical profile and approach to management in the pediatric population.
, , 96
Evaluation of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients.
, , 98
Stress-rest myocardial perfusion SPECT for functional assessment of coronary arteries with anomalous origin or course.
, 99
Clinical features of prognosis of Japanese patients with anomalous origin of the coronary artery.
; however, it must be emphasized that a normal stress test, nuclear perfusion scan, or stress echocardiogram is, at best, incompletely reassuring. These studies have a low negative predictive value, and are most helpful only if they are positive. Recommendations on Diagnostic Imaging
Section III: TreatmentOverviewMost would agree that activity restriction and surgical intervention is indicated in any patient with AAOCA who presents with signs and/or symptoms of myocardial ischemia, or has inducible ischemic changes with exercise testing. The treatment dilemma occurs when this diagnosis is made in the asymptomatic patient, especially the patient with AAORCA, as provocative testing is often negative. Treatment options for AAOCA include surgical and nonsurgical interventions and/or medical management. Surgical InterventionThe clinical indication for intervention, even in those who are asymptomatic, is based on the calculated risk of SCD. Although an intervention is not warranted in most cases of coronary anomalies, there are some instances in which intervention may be warranted, including syncope associated with documented or reasonably suspected ventricular arrhythmia (nonvagally mediated), high-risk ambient ventricular arrhythmias, chest pain consistent with angina, aborted sudden death/cardiac arrest, and evidence of ischemia on provocative testing. , 102
ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease).
In those with interarterial AAOLCA, most children ages 10 and older are referred for surgical intervention, even if asymptomatic, due to the significantly increased risk of SCD with this anomaly, especially with exercise. 103
Congenital Heart Surgeons Society AAOCA Working Group. Anomalous aortic origin of a coronary artery: a report from the Congenital Heart Surgeons Society Registry.
In contrast, there is far less agreement about whether patients with AAORCA without symptoms should undergo operation. Until recently, American College of Cardiology (ACC) and American Heart Association (AHA) guidelines have suggested avoidance of athletic activity for all patients with AAOCA and so for asymptomatic young patients with AAORCA, surgery has sometimes been performed to permit them to participate in competitive athletics. 104
ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in collaboration with the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons.
, 105
Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 4: congenital heart disease: a scientific statement from the American Heart Association and American College of Cardiology.
The recent AHA/ACC Scientific Statement now differentiates between the much higher-risk interarterial AAOLCA and the lower-risk interarterial AAORCA, with the potential for those with AAORCA who are asymptomatic to return to competitive sports after a complete evaluation. 105
Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 4: congenital heart disease: a scientific statement from the American Heart Association and American College of Cardiology.
AAOLCA with intraseptal, prepulmonic, or retroaortic courses are generally considered benign with only rare case reports of ischemia. 106
Clinical, angiographic, and hemodynamic findings in patients with anomalous origin of the coronary arteries.
, , 108
Anomalous origin of left main cononary artery from anterior sinus of Valsalva with myocardial infarction.
These patients are generally not referred for surgery and are not exercise-restricted, but should have a stress test as part of preparticipation clearance for competitive athletics. Once the decision to perform surgery has been made, several surgical procedures are available. These various approaches have not been compared in any rigorous fashion, and so the superiority of any one remains speculative. Each type of operation is performed to address 1 or more of the proposed mechanisms of myocardial ischemia in patients with AAOCA, as discussed previously. Specific surgical procedures.UnroofingAmong the various surgical options, perhaps the most technically and conceptually simple is the so-called “unroofing” procedure. It is the procedure of choice for interarterial, intramural AAOCA in the young patient. 109
Repair of anomalous aortic origin of a coronary artery in 113 patients: a Congenital Heart Surgeons' Society report.
, 110
Anomalous origin of the left coronary artery from the anterior aortic sinus: a potential cause of sudden death. Anatomic characterization and surgical treatment.
The operation is accomplished via an anterior aortotomy and consists of incising the common wall between the aorta and intramural segment of the anomalous coronary artery (Figure 3). This incision has several salutary consequences in that it relocates the functional orifice to the appropriate sinus, enlarges the orifice significantly, eliminates the intramural component of the artery, and eliminates the portion of the vessel that lies between the great arteries. Figure 3The unroofing technique in a patient with anomalous origin of the left coronary artery from the right sinus of Valsalva with an intramural course. The normally positioned right coronary artery orifice and the anomalous left coronary artery orifice can both be seen arising from the right sinus (A). The intramural segment of the anomalous coronary is unroofed and the common wall debulked (B) to create a neo-orifice in the left sinus (C). Reprinted with permission. 53
Prospective echocardiographic diagnosis and surgical repair of anomalous origin of a coronary artery from the opposite sinus with an interarterial course.
There are 3 particular pitfalls to be avoided in the performance of the operation. The intramural course typically runs behind the intracoronary commissure, which might be damaged or distorted by the unroofing incision, potentially causing aortic insufficiency. Strategies for approaching this anatomic variant include resuspension of the commissure if it has been injured or creating a neo-ostium of the anomalous vessel by unroofing only that portion which is not behind the commissure (Figure 4). 53
Prospective echocardiographic diagnosis and surgical repair of anomalous origin of a coronary artery from the opposite sinus with an interarterial course.
, Another potential difficulty with the unroofing procedure is the exposure of the layers of the aortic wall to systemic pressure at the site of the neo-ostium, creating the possibility for a localized dissection, which might occlude the coronary artery. This can be prevented by the placement of fine “tacking” sutures to approximate the layers of the aortic wall at the neo-ostium. An additional hazard of unroofing is seen with overly aggressive unroofing, whereby the incision is inadvertently carried through the aortic or coronary wall in an area in which the coronary is not actually intramural. This is particularly easy if the intramural component of the vessel is very short. If not recognized, this can result in troublesome bleeding, which requires very precisely placed sutures for repair, possibly necessitating an additional period of cardioplegic arrest. Clearly the best approach to this complication is prevention. Figure 4Unroofing procedure in which a neo-ostium was created without taking down the intercoronary commissure. A, The left coronary artery arises from the right sinus of Valsalva with an intramural course. The site for creation of a neo-ostium is identified by passing an instrument into the coronary (B). The anomalous coronary is unroofed into the appropriate sinus (C). Because the commissure between the left and right sinuses is not disrupted, this may decrease the risk of aortic valve regurgitation. A neo-ostium is created in that sinus at the point at which the artery leaves the aortic wall. Reprinted with permission.
Pulmonary artery translocationThis operation is based on the concept that the anomalous vessel is compressed or pinched between the great arteries in AAOCA, and therefore the goal of the operation is to reposition the main pulmonary artery (Figure 5). , 113
Surgical repair of anomalous aortic origin of a coronary artery.
This technique is mainly used when the anomalous coronary is not intramural but the ostia are in close approximation or if there is a single coronary artery in the presence of ischemia. This procedure consists of an initial meticulous dissection to permit full mobilization of the proximal pulmonary root. In one version of the operation, the main pulmonary artery is transected, the pulmonary confluence is patched, and the main pulmonary artery is anastomosed to a “neo-confluence” location created by making an incision well out onto the left pulmonary artery. In effect, this moves the course of the main pulmonary artery leftward, preventing it from compressing the anomalous coronary. An alternative version of the operation is accomplished by dividing the proximal right pulmonary artery at its origin and then transposing it anterior to the aorta, where it is re-anastomosed to its original site. This is usually accomplished by a tissue-to-tissue posterior wall suture line, with a generous augmentation patch placed anteriorly to prevent stenosis in the stretched right pulmonary artery. This version of the operation has the effect of moving the main pulmonary artery anteriorly and away from the anomalous coronary artery. Figure 5Illustration of the pulmonary artery translocation procedure. The distal main pulmonary artery is transected (A) and then relocated into the left pulmonary artery (B) to prevent compression of the anomalous coronary artery between the 2 great vessels. An alternative method involves transection of the right pulmonary artery at its origin (C). The right pulmonary artery is then brought anterior to the aorta and re-anastomosed to the main pulmonary with anterior patch augmentation (D). Ao, Aorta; PA, pulmonary artery. Reprinted with permission.
Reimplantation (ostial translocation)Reimplantation is perhaps most useful when there is little or no intramural component of the anomalous coronary and there are 2 separate ostia. , , 115
Surgery for anomalous origin of the left main coronary artery from the right sinus of Valsalva, in association with left main stenosis.
, , In this operation, the anomalous vessel is mobilized over its proximal course. Ideally, a few millimeters of aortic wall containing the ostium of the anomalous coronary is excised from its abnormal location, and then reimplanted in the correct sinus of Valsalva (Figure 6). Sometimes a patch of autologous pericardium is used to enlarge the connection. It is somewhat technically challenging in that it requires full mobilization of the anomalous vessel to avoid kinking as well as very precise patching and reimplantation. Figure 6Direct reimplantation of an anomalous right coronary artery arising from the left sinus of Valsalva. A button of aortic sinus containing the anomalous coronary is excised and mobilized (A). The anomalous coronary is then reimplanted into the right sinus of Valsalva (B). RCA, Right coronary artery; L, left; R, right. Reprinted with permission.
OstioplastyIn the ostial approach, a neo-ostium is created for the anomalous vessel in the sinus from which it would normally have exited. An incision is created in the aortic sinus with a matching incision in the coronary artery away from the aortic wall. By joining these incisions and augmenting the opened areas with a patch, typically of autologous pericardium, a neo-ostium is fashioned (Figure 7), and the course of the artery is no longer either intramural or between the great vessels. , , The operation is facilitated by transection of the main pulmonary artery to optimize exposure, and requires meticulous reconstruction of the coronary artery itself. Of all of the surgical options, this is therefore probably the most technically challenging. Figure 7An ostioplasty for anomalous origin of the left coronary artery from the right sinus of Valsalva. A, The aorta and pulmonary artery have been transected above the commissures. The eccentric slit orifice in the opposite sinus of Valsalva, proximal intramural segment, nonorthogonal angle of exit, and interarterial course of the left coronary artery are demonstrated. B, The left coronary artery has been opened from its origin in the aorta to the bifurcation. C, An autologous pericardial patch is sutured into the coronary artery and aortic wall. Reprinted with permission.
Bypass graftingAn indirect approach for AAOCA is to use standard coronary artery bypass techniques, with either a mammary artery or saphenous vein conduit. 121
Surgical treatment of coronary artery anomalies: report of a 37 1/2-year experience at the Texas Heart Institute.
This approach has generally been unsatisfactory because flow in the anomalous vessel is typically only compromised during periods of stress or exercise, so that there is substantial competitive flow the vast majority of the time. The patency of a bypass graft in such a setting is likely to be disappointing. Ligation of the native vessel at its origin has been advocated by some as a solution to the issue of competitive flow, but concern has also been raised that a native mammary artery may have inadequate flow early on to replace all of the flow produced by a nonstenotic native coronary artery. Therefore, bypass grafting has a very limited role for AAOCA, and should probably be limited to settings in which the anomaly is accompanied by significant atherosclerotic narrowing or in which an alternative approach has been technically unsatisfactory. Summary of surgical approaches to AAOCAIn summary, surgical address of AAOCA may take 1 of several forms. For patients with significant intramural length of the anomalous vessel, unroofing is generally the preferred procedure. For patients with a very short or minimal intramural course, reimplantation or ostial reconstruction would be more appropriate. If the AAOCA is accompanied by atherosclerotic narrowing or if an alternative reconstruction technique has failed, coronary bypass grafting is reasonable. Pulmonary artery translocation techniques may be used to supplement any primary reconstruction, and are relatively low risk and technically simple adjuncts to other procedures. The risk of surgery for AAOCA in published series is extremely low, with excellent intermediate-term survival. 32
Anomalous origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course: clinical profile and approach to management in the pediatric population.
, , 56
Anomalous aortic origin of a coronary artery: preoperative diagnosis and surgical planning.
, , , , , , 96
Evaluation of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients.
, 105
Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 4: congenital heart disease: a scientific statement from the American Heart Association and American College of Cardiology.
, , , 113
Surgical repair of anomalous aortic origin of a coronary artery.
, , 123
Surgical repair of 115 patients with anomalous aortic origin of a coronary artery from a single institution.
, 124
Anomalous aortic origin of the left coronary artery: in-hospital cardiac arrest and death despite bed rest.
Nevertheless, despite a patent coronary ostium, 1 study found that subclinical changes suggestive of ischemia occur on stress testing in more than one-third of postoperative patients. 96
Evaluation of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients.
Furthermore, although the surgical procedures may remove the hypothesized mechanism of ischemia leading to SCD, the long-term impact of surgery on the coronary arteries is unknown. Specifically, we do not know if these procedures place the patient at long-term risk of coronary stenosis due to scarring or accelerated atherosclerosis. Ongoing assessment of patients who have undergone surgery for AAOCA is essential to define the risks both short- and long-term. Percutaneous Coronary InterventionBefore the development of the field of percutaneous coronary interventions (PCI), management of symptomatic or high-risk coronary arteries with anomalous origins, with or without superimposed atherosclerotic lesions, was limited to surgery and medical therapy directed at superimposed coronary vasospasm. The development of PCI technology has added the strategy of stenting the anomalous vessels, based on their anatomic take-off anatomy and coexisting lesions, with or without vasospasm. It has been suggested that intravascular ultrasound technology aids in the PCI approach to identifying and intervening for an ischemic burden associated with AAORCA, especially in those with a partial intramural course and proximal intramural stenosis. 125
Origin of the right coronary artery from the opposite sinus of Valsalva in adults: characterization by intravascular ultrasonography at baseline and after stent angioplasty.
There are no large-scale studies or registries comparing outcomes of surgery versus PCI, leaving evaluation limited to objective ischemic burden testing before and after interventions. Furthermore, long-term follow-up data are limited regarding this procedure in adults. Due to safety issues with stenting anomalous coronary arteries in growing children, this procedure is not advisable in the pediatric population, but may be considered in select cases in the adult population. Medical TherapyBeta-blockersThere are a small number of case reports using beta-blockers in adults to treat AAOCA. 126
Successful medical management of a patient with an anomalous right coronary artery who declined surgery.
, The single largest series in the literature described 56 adult patients (average age 55.9 years) with beta-blockade and reported no episodes of SCD over a 5-year period. 99
Clinical features of prognosis of Japanese patients with anomalous origin of the coronary artery.
In this study, however, there were no patients with AAOLCA and no patients younger than 30, both variables known to increase the risk of sudden death in patients with AAOCA. There are no reports of the use of beta-blockers in patients with AAOCA younger than 30 years. Presently, there does not appear to be adequate data to justify medical therapy for AAOCA. Exercise restriction from competitive athleticsIn the young patient, exercise restriction from competitive athletics is the most common nonsurgical strategy. The recommendation to avoid competitive athletics in patients with AAOCA is based on 2 observations: (1) the mortality risk associated with AAOCA is high, and (2) sudden death in patients with AAOCA occurs most frequently during or after peak exercise. 6
Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles.
, 18
Anomalous right or left coronary artery from the contralateral coronary sinus: “high-risk” abnormalities in the initial coronary artery course and heterogeneous clinical outcomes.
, , 22
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.
, , However, because children should remain active and discouraging exercise may lead to other issues with long-term cardiovascular health, asymptomatic children are encouraged to participate in physical education class and other recreational activities. In contrast to previous recommendations, the current recommendations by the AHA/ACC permit individuals with unrepaired AAORCA who are without symptoms or a positive exercise stress test to participate in competitive sports. 105
Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 4: congenital heart disease: a scientific statement from the American Heart Association and American College of Cardiology.
, It is important to note, however, that as of this report, there are no short- or long-term follow-up studies on unoperated patients with AAOCA who have been observed and restricted from competitive sports. The guidelines are generally based on Level of Evidence C, expert consensus opinions, in the absence of reliable objective clinical or epidemiological data. The one exception is surgery for interarterial AAOLCA, for which intervention is based on Level of Evidence B. To better address this issue, the Congenital Heart Surgeons' Society has established a Registry of Anomalous Aortic Origin of the Coronary Artery. 129
The registry of anomalous aortic origin of the coronary artery of the Congenital Heart Surgeons' Society.
The overall purpose of the registry is to determine the outcome of surgical intervention versus observation in children and young adults with AAOCA, and to describe the natural and “unnatural” history of these patients over the course of their lifetime. Observation without exercise restrictionIf the cumulative risk for interarterial AAORCA is estimated at 0.2%, is it safer to observe than to recommend surgery? We must also take into account the risks of surgical intervention, the lack of long-term follow-up for these patients, and the few scattered reports of postoperative sudden death, ischemia, and other complications from surgery. , 96
Evaluation of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients.
, , We agree with the current AHA/ACC guidelines in that participation in competitive sports may be a reasonable option for the asymptomatic patient with AAORCA without evidence of inducible ischemia, as this appears to be a more benign lesion than previously recognized. It is essential that we have a clear definition of “symptoms.” Palpitations, chest pain, and even presyncope are common in the pediatric population and rarely have a cardiac etiology, whereas frank syncope and true anginalike chest pain with exercise are much less common and could be considered related to the coronary anomaly. Thus, even in the symptomatic patient, if all provocative testing for ischemia is negative, especially in the case in which the symptoms are replicated during the test and the test is normal, there is little reason to limit these individuals. In the absence of inducible ischemia on testing, there should be convincing historical evidence that the symptoms are likely due to ischemia or arrhythmias before restricting the individual from competitive athletics. The decision to participate in competitive or high-intensity recreational sports should include a thorough family discussion and counseling regarding the risks and benefits of observation versus surgical management of this lesion. We recommend that automated external defibrillators (AEDs) should be available and accessible for sporting practices and competition. 131
Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 12: emergency action plans, resuscitation, cardiopulmonary resuscitation, and automated external defibrillators: a scientific statement from the American Heart Association and American College of Cardiology.
, , , , 135
Prospective assessment of integrating the existing emergency medical system with automated external defibrillators fully operated by volunteers and laypersons for out-of-hospital cardiac arrest: the Brescia Early Defibrillation Study (BEDS).
, , 137
Chest compression-only cardiopulmonary resuscitation for out-of-hospital cardiac arrest with public-access defibrillation: a nationwide cohort study.
, 138
Public access defibrillation improved the outcome after out-of-hospital cardiac arrest in school-age children: a nationwide, population-based, Utstein registry study in Japan.
, , 140
Impact of onsite or dispatched automated external defibrillator use on survival after out-of-hospital cardiac arrest.
, 141
Out-of-hospital early defibrillation successfully challenges sudden cardiac arrest: the Piacenza Progetto Vita project.
, 142
Outcomes from sudden cardiac arrest in US high schools: a 2-year prospective study from the National Registry for AED Use in Sports.
, 143
Effectiveness of emergency response planning for sudden cardiac arrest in United States high schools with automated external defibrillators.
The immediate availability of AEDs during competitive athletics is also recommended in the scientific statement concerning eligibility and disqualification of competitive athletes with cardiovascular abnormalities by the AHA and ACC. 144
Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 12: emergency action plans, resuscitation, cardiopulmonary resuscitation, and automated external defibrillators: a scientific statement from the American Heart Association and American College of Cardiology.
For those with interarterial AAOLCA, even if asymptomatic, surgical repair is usually recommended, However, if after consultation with the family, they opt for no surgical intervention, the patient with AAOLCA should be restricted from competitive athletics. Recommendations on Treatment
Section IV: Follow-upOngoing RiskThe follow-up for patients with AAOCA will depend somewhat on the management chosen; however, all patients should be followed by a cardiologist for their lifetime. This should include a transition from a pediatric to an adult cardiologist, if necessary. Lifelong follow-up is especially important for those who undergo surgical repair, as long-term outcomes from surgical repair are largely unknown. Short- and mid-term complications have been noted, including mild aortic valve insufficiency, severe aortic valve insufficiency requiring aortic valve replacement, pericardial effusions, and ischemic changes on postoperative provocative testing. 96
Evaluation of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients.
, 109
Repair of anomalous aortic origin of a coronary artery in 113 patients: a Congenital Heart Surgeons' Society report.
, , Postoperative death has been reported rarely in the literature in both the pediatric and adult age groups. , 146
Sudden cardiac death after repair of anomalous origin of left coronary artery from right sinus of Valsalva with an interarterial course: case report and review of the literature.
, 147
Anomalous aortic origin of a coronary artery with an interarterial course: understanding current management strategies in children and young adults.
The authors are aware of a handful of other pediatric deaths postoperatively that have not been published. It is interesting to note that, at least in the young, if a patient presents with SCD or SCA and survives the surgery, he or she may still be at increased risk for an SCD event once the patient returns to sport. 146
Sudden cardiac death after repair of anomalous origin of left coronary artery from right sinus of Valsalva with an interarterial course: case report and review of the literature.
Those who have undergone surgery will need close follow-up in the initial postoperative period, but will be able to lengthen the interval between visits after the first postoperative year. According to the recent AHA/ACC guidelines, these patients may return to competitive sports at least 3 months postoperatively and if an exercise stress test reveals no evidence of myocardial ischemia or ventricular arrhythmias. 104
ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in collaboration with the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons.
, 105
Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 4: congenital heart disease: a scientific statement from the American Heart Association and American College of Cardiology.
However, those who present with true aborted SCD appear to remain at increased risk, even after surgical repair. 146
Sudden cardiac death after repair of anomalous origin of left coronary artery from right sinus of Valsalva with an interarterial course: case report and review of the literature.
We would recommend those patients with true SCA or aborted SCD should not return to competitive athletics for at least 1 year postoperatively and then only return to competitive athletics if they are free of symptoms and have a negative exercise stress test. Those managed conservatively with exercise restriction will need follow-up annually. Those patients who are not exercise-restricted should be seen annually, especially if they are participating in competitive athletics. Finally, when there is a family history of clustering of SCD as a first manifestation of any ischemic event, more specific counseling of risk-versus-benefit should be undertaken with both the athlete and family so they can make an informed decision about participation. For further details, please refer to Table 2, Table 3, Table 4, which are suggested protocols that we have created for follow-up of patients with AAOCA, based on their management. Table 2Recommended follow-up for AAOCA, surgical repair AAOCA, Anomalous aortic origin of a coronary artery; ECG, electrocardiogram; MRI, magnetic resonance imaging.
Table 4Follow-up and activity recommendations for AAOCA, restricted from competitive athletics
Recommendations on Follow-up
Section V: Gaps in KnowledgeOne major gap in knowledge regarding AAOCA is our ability to adequately risk stratify patients for surgery versus observation, notably in the asymptomatic young person with AAORCA. Although there are suspected anatomic and physiologic reasons for myocardial ischemia and/or potentially lethal arrhythmias, we are still unable to distinguish which person is at high risk for ischemia and should undergo surgery from the individual who will remain asymptomatic and may be allowed to participate in competitive sports. Another gap arises from our lack of long-term follow-up of patients after surgical repair. Short- and medium-term results are encouraging; however, there are reports of issues, such as new aortic valve regurgitation, that will need to be followed over time. Section VI: Summary and RecommendationsSummaryAAOCA with an intramural course is associated with SCD, due to ischemia and arrhythmias. 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 that there is net benefit to the therapy. Although we acknowledge there is still much to be learned with this entity, the guidelines put forth in this article are based on best practice and knowledge regarding the risk of SCD that we have to date. Recommendations
WebcastConflict of Interest StatementAuthors have nothing to disclose with regard to commercial support. References
Article InfoPublication HistoryPublished online: February 03, 2017 Accepted: June 29, 2016 Received in revised form: June 27, 2016 Received: April 14, 2016 IdentificationDOI: https://doi.org/10.1016/j.jtcvs.2016.06.066 Copyright© 2017 by The American Association for Thoracic Surgery User LicenseElsevier user license |How you can reuse PermittedFor non-commercial purposes:
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Related ArticlesHow serious is anomalous right coronary artery?Anomalous right coronary arteries (ARCA) are extremely rare in general population. Although mostly asymptomatic and recognized incidentally on cardiac catheterizations, they can be catastrophic and can cause sudden cardiac death.
Can you live with anomalous coronary artery?While most people with an anomalous coronary artery aren't in danger, the problem can be very dangerous for some. Sometimes people don't have symptoms until they have a sudden cardiac arrest. Fortunately, several surgical options can protect you from sudden death and help you have a normal life.
Which anomalous coronary artery course should be considered for surgery?Indications for surgery
Asymptomatic individuals with the left main coronary artery arising from the right sinus of Valsalva should be offered surgery (class 1, level of evidence B).
What is anomalous origin of the right coronary artery?Anomalous aortic origin of a coronary artery (AAOCA) is a rare condition in which both of the coronary arteries arise from the same aortic sinus. In a normal heart, the left coronary artery arises from the left aortic sinus and the right coronary artery from the right aortic sinus.
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Anomalous right coronary artery between aorta and pulmonary artery
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