The physical examination and resting ECG in a patient with AAOCA will most likely be normal A high index of suspicion is necessary in patients complaining of exercise-induced symptoms suggesting myocardial ischemia or arrhythmia in order to reliably make the correct diagnosis In particular, syncope with exercise or patients presenting with aborted sudden cardiac death should have their coronary artery origins carefully evaluated Diagnostic Testing Although unlikely to demonstrate abnormalities, a resting ECG should be obtained in any child who is being evaluated for a coronary anomaly to evaluate for arrhythmias or evidence of prior or evolving myocardial infarction Echocardiography A transthoracic echocardiogram is the preferred initial diagnostic test due to its ease of use in children even without sedation, widespread availability, lack of radiation, and cost effectiveness Transthoracic echocardiography should confirm normal cardiac anatomy and evaluate ventricular function In particular, wall motion should be carefully assessed for regional wall motion abnormalities, which may indicate a history of ischemia Coronary arterial origins, and the proximal course of the coronary arteries, should be evaluated carefully by twodimensional imaging and color Doppler interrogation In the majority of children, the origins and proximal course of the coronary arteries can be visualized Color Doppler interrogation is particularly important in identifying the proximal course of intramural anomalous coronary arteries because twodimensional imaging alone can be deceptive (Fig 46.18) In patients for whom concern remains for an anomalous coronary artery that cannot be clearly seen by echocardiogram, advanced imaging modalities are typically used In many centers, confirmatory computed tomographic angiography (CTA) or cardiac MRI is performed in most children when an AAOCA is suspected (Fig 46.19, Video 46.4) FIG 46.18 Echocardiographic image in the short axis at the base of the heart showing the right coronary artery arising from the left sinus of Valsalva in two-dimensional imaging (left) and color Doppler interrogation (right) FIG 46.19 Computed tomography image of an anomalous right coronary artery from the left sinus of Valsalva with suspected intramural course AoV, Aortic valve; LA, left auricle; LAD, left anterior descending; LV, left ventricle; RCA, right coronary artery; RV, right ventricle; RVOT, right ventricular outflow tract; VS, ventricular septum Computed Tomographic Angiography and Magnetic Resonance Imaging Coronary CTA is an excellent choice for diagnostic confirmation of suspected coronary artery anomalies It provides detailed information regarding the shape of the coronary arterial orifice, the angle of the coronary artery take-off, and the proximal and distal course.44 Using three-dimensional reconstruction, the intraluminal anatomy and the location of the aortic valvar commissure relative to the anomalous coronary artery can be shown, which aids in surgical planning (Video 46.5).45,46 Given advances in CT technology, current-generation scanners can typically obtain the necessary data with high resolution within one breath hold and sometimes within one to three cardiac cycles.44–47 However, CTA does have limitations, including radiation, the need for heart rate control, timing of scan relative to intravenous contrast administration, and minimization of patient motion Cardiac MRI can accurately detect anomalous coronary artery origins without the use of radiation and, similar to CTA, can use three-dimensional reconstruction techniques to evaluate the intraluminal anatomy.48–50 However, cardiac MRI is limited by the amount of time needed to obtain the images, thus requiring sedation in younger children Whether CT or MRI is used to confirm the anomalous coronary artery anatomy depends on the provider and/or institution Cardiac catheterization with angiography of the coronary arteries has limited use in the diagnosis of AAOCA in children but has been used in latepresenting adult patients to evaluate for atherosclerotic coronary artery disease prior to surgical intervention Cardiopulmonary Exercise Stress Testing After establishing that the patient has AAOCA, cardiopulmonary exercise testing (CPET) is almost always used to evaluate for ischemia and inducible arrhythmias Because exercise stress testing alone is not a sensitive test in children for myocardial ischemia, it is recommended that an additional imaging modality be used to optimize the sensitivity of stress testing.51,52 Depending on the institutional preference, this can be a nuclear perfusion scan or stress echocardiogram Postoperatively, CPET is used prior to allowing the patient to return to competitive play.53 However, it should be noted that basing management decisions on a CPET can be difficult because ischemia in AAOCA is typically intermittent and the positive predictive value of a positive test in this population is quite low.54 Surgical Intervention Surgical intervention is indicated in any patient with AAOLCA or AAORCA with an interarterial course who has signs and/or symptoms of myocardial