Anomalous origination of a coronary artery from the opposite sinus (ACAOS) is estimated to be present in 0.2–2.0% of the population. In the majority of individuals, ACAOS has no hemodynamic or prognostic implications, but in a minority of cases, typically where the anomalous coronary artery takes an interarterial course to reach its correct myocardial territory, it can precipitate ischemia and sudden cardiac death (SCD). With the growing use of CT coronary angiography (CTCA) in the investigation of ischemic heart disease, we can expect increasing rates of incidental detection of this anomaly. Although CTCA and magnetic resonance coronary angiography can effectively characterize these lesions anatomically, they fail to describe and quantitatively assess the basic defect that leads to coronary insufficiency, such as mural intussusception. The key challenge lies in the identification of which patients are at risk of SCD and, therefore, who should be offered corrective surgical or (potentially) percutaneous intervention. Conventional, noninvasive stress testing has limited sensitivity, but emerging, invasive stress tests, which utilize intravascular ultrasonography and measurements of fractional flow reserve, show the potential to provide more-accurate hemodynamic and prognostic assessment.
Anomalous origination of a coronary artery from the opposite sinus (ACAOS) is a recognized cause of sudden cardiac death (SCD), typically associated with exercise
Insights from intravascular ultrasonography (IVUS) studies suggest that the pathophysiological mechanism by which ACAOS causes SCD involves systolic compression of the anomalous artery within the aortic wall
Cardiac catheterization was regarded as the gold standard for diagnosis and anatomical characterization of ACAOS, but has been superseded by CT and magnetic resonance coronary angiography
Standard, noninvasive stress tests have limited sensitivity in predicting the risk of SCD in patients with ACAOS
Invasive stress tests using IVUS and measurements of fractional flow reserve might provide a means of establishing the hemodynamic and prognostic significance of ACAOS
Case reports of successful percutaneous coronary intervention for treatment of ACAOS exist, but surgery remains the first-line treatment of choice
Subscribe to Journal
Get full journal access for 1 year
only $17.75 per issue
All prices are NET prices.
VAT will be added later in the checkout.
Rent or Buy article
Get time limited or full article access on ReadCube.
All prices are NET prices.
Angelini, P., Velasco, J. A. & Flamm, S. Coronary anomalies: incidence, pathophysiology, and clinical relevance. Circulation 105, 2449–2454 (2002).
Hauser, M. Congenital anomalies of the coronary arteries. Heart 91, 1240–1245 (2005).
Angelini, P. Coronary artery anomalies: an entity in search of an identity. Circulation 115, 1296–1305 (2007).
Sundaram, B., Kreml, R. & Patel, S. Imaging of coronary artery anomalies. Radiol. Clin. North Am. 48, 711–727 (2010).
Angelini, P. in Sports Cardiology (ed. Lawless, C. E.) 277–298 (Springer, New York, 2010).
Cooper, A. et al. Chest pain of recent onset: Assessment and diagnosis of recent onset chest pain or discomfort of suspected cardiac origin. Full guideline. National Institute for Health and Clinical Excellence [online], (2010).
Edwards, J. E. Anomalous coronary arteries with special reference to arteriovenous-like communications. Circulation 17, 1001–1006 (1958).
Blake, H. A. et al. Coronary artery anomalies. Circulation 30, 927–934 (1964).
Cheitlin, 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 50, 780–787 (1974).
Yamanaka, O. & Hobbs, R. E. Coronary artery anomalies in 126,595 patients undergoing coronary angiography. Cathet. Cardiovasc. Diagn. 21, 28–40 (1990).
Angelini, P., Villason, S., Chan, A. V. & Diez J. G. in Coronary Artery Anomalies (ed. Angelini, P.) 27–79 (Lippincott Williams & Wilkins, Chicago, 1999).
Kim, S. Y. et al. Coronary artery anomalies: classification and ECG-gated multi-detector row CT findings with angiographic correlation. Radiographics 26, 317–333 (2006).
Frommelt, P. C., Sheridan, D. C., Berger, S., Frommelt, M. A. & Tweddell, J. S. Ten-year experience with surgical unroofing of anomalous aortic origin of a coronary artery from the opposite sinus with an interarterial course. J. Thorac Cardiovasc. Surg. http://dx.doi.org/10.1016/j.jtcvs.2011.02.004.
Cheitlin, M. D. & MacGregor, J. Congenital anomalies of coronary arteries: role in the pathogenesis of sudden cardiac death. Herz. 34, 268–279 (2009).
Yildiz, A. et al. Prevalence of coronary artery anomalies in 12,457 adult patients who underwent coronary angiography. Clin. Cardiol. 33, E60–E64 (2010).
Correia, E. et al. Prevalence of anomalous origin of coronary arteries: a retrospective study in a Portuguese population. Rev. Port. Cardiol. 29, 221–229 (2010).
Eid, A. H., Itani, Z., Al-Tannir, M., Sayegh, S. & Samaha, A. Primary congenital anomalies of the coronary arteries and relation to atherosclerosis: an angiographic study in Lebanon. J. Cardiothorac. Surg. 4, 58 (2009).
Ouali, S. et al. Congenital anomalous aortic origins of the coronary arteries in adults: a Tunisian coronary arteriography study. Arch. Cardiovasc. Dis. 102, 201–208 (2009).
Aydinlar, A. et al. Primary congenital anomalies of the coronary arteries: a coronary arteriographic study in Western Turkey. Int. Heart J. 46, 97–103 (2005).
Mavi, A. et al. Frequency in the anomalous origin of the left main coronary artery with angiography in a Turkish population. Acta Med. Okayama 58, 17–22 (2004).
Rigatelli, G. et al. Congenital coronary artery anomalies angiographic classification revisited. Int. J. Cardiovasc. Imaging 19, 361–366 (2003).
Harikrishnan, S. et al. Congenital coronary anomalies of origin and distribution in adults: a coronary arteriographic study. Indian Heart J. 54, 271–275 (2002).
Ayalp, R., Mavi, A., Serçelik, A., Batyraliev, T. & Gümüsburun, E. Frequency in the anomalous origin of the right coronary artery with angiography in a Turkish population. Int. J. Cardiol. 82, 253–257 (2002).
Garg, N., Tewari, S., Kapoor, A., Gupta, D. K. & Sinha, N. Primary congenital anomalies of the coronary arteries: a coronary: arteriographic study. Int. J. Cardiol. 74, 39–46 (2000).
Kardos, A. et al. Epidemiology of congenital coronary artery anomalies: a coronary arteriography study on a central European population. Cathet. Cardiovasc. Diagn. 42, 270–275 (1997).
Wilkins, C. E. et al. Coronary artery anomalies: a review of more than 10,000 patients from the Clayton Cardiovascular Laboratories. Tex. Heart Inst. J. 15, 166–173 (1988).
Zhang, L. J. et al. Incidence of anomalous origin of coronary artery in 1879 Chinese adults on dual-source CT angiography. Neth. Heart J. 18, 466–470 (2010).
Cheng, Z. et al. Detection of coronary artery anomalies by dual-source CT coronary angiography. Clin. Radiol. 65, 815–822 (2010).
von Ziegler, F. Visualization of anomalous origin and course of coronary arteries in 748 consecutive symptomatic patients by 64-slice computed tomography angiography. BMC Cardiovasc. Disord. 9, 54 (2009).
Kos¸ar, P., Ergun, E., Oztürk, C. & Kos¸ar, U. Anatomic variations and anomalies of the coronary arteries: 64-slice CT angiographic appearance. Diagn. Interv. Radiol. 15, 275–283 (2009).
Duran, C. et al. Remarkable anatomic anomalies of coronary arteries and their clinical importance: a multidetector computed tomography angiographic study. J. Comput. Assist. Tomogr. 30, 939–948 (2006).
Sato, Y. et al. Detection of anomalous origins of the coronary artery by means of multislice computed tomography. Circ. J. 69, 320–324 (2005).
Schmitt, R. et al. Congenital anomalies of the coronary arteries: imaging with contrast-enhanced, multidetector computed tomography. Eur. Radiol. 15, 1110–1121 (2005).
Maron, B. J. et al. Sudden death in young competitive athletes: clinical, demographic, and pathological profiles. JAMA 276, 199–204 (1996).
Eckart, R. E. et al. Sudden death in young adults: a 25-year review of autopsies in military recruits. Ann. Intern. Med. 141, 829–834 (2004).
Basso, 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. 35, 1493–1501 (2000).
Taylor, A. J., Rogan, K. M. & Virmani, R. Sudden cardiac death associated with isolated congenital coronary artery anomalies. J. Am. Coll. Cardiol. 20, 640–647 (1992).
Kragel, 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. 62, 771–777 (1988).
Roberts, 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. 49, 863–868 (1982).
Cox, I. D., Bunce, N. & Fluck, D. S. Failed sudden cardiac death in a patient with an anomalous origin of the right coronary artery. Circulation 102, 1461–1462 (2000).
Chaitman, B. R., Lesperance, J., Saltiel, J. & Bourassa, M. G. Clinical, angiographic, and hemodynamic findings in patients with anomalous origin of the coronary arteries. Circulation 53, 122–131 (1976).
Frommelt, P. C., Frommelt, M. A., Tweddell, J. S. & Jaquiss, R. D. Prospective echocardiographic diagnosis and surgical repair of anomalous origin of a coronary artery from the opposite sinus with an interarterial course. J. Am. Coll. Cardiol. 42, 148–154 (2003).
Angelini, P., Walmsley, R. P., Libreros, A. & Ott, D. A. Symptomatic anomalous origination of the left Coronary artery from the opposite sinus of Valsalva: Clinical presentations, diagnosis, and surgical repair. Tex. Heart Inst. J. 33, 171–179 (2006).
Angelini, P., Walmsley, R., Cheong, B. Y. & Ott, D. A. Left main coronary artery originating from the proper sinus but with acute angulation and an intramural course, leading to critical stenosis. Tex. Heart Inst. J. 37, 221–225 (2010).
Davis, 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. 37, 593–597 (2001).
Manghat, N. E., Morgan-Hughes, G. J., Marshall, A. J. & Roobottom, C. A. Multidetector row computed tomography: imaging congenital coronary artery anomalies in adults. Heart 91, 1515–1522 (2005).
Bluemke, D. A. et al. Noninvasive coronary artery imaging: magnetic resonance angiography and multidetector computed tomography angiography: a scientific statement from the American Heart Association Committee on Cardiovascular Imaging and Intervention of the Council on Cardiovascular Radiology and Intervention, and the Councils on Clinical Cardiology and Cardiovascular Disease in the Young. Circulation 118, 586–606 (2008).
Zeina, A. R., Blinder, J., Sharif, D., Rosenschein, U. & Barmeir, E. Congenital coronary artery anomalies in adults: non-invasive assessment with multidetector CT. Br. J. Radiol. 82, 254–261 (2009).
Schroeder, S. et al. Cardiac computed tomography: indications, applications, limitations, and training requirements: report of a Writing Group deployed by the Working Group Nuclear Cardiology and Cardiac CT of the European Society of Cardiology and the European Council of Nuclear Cardiology. Eur. Heart J. 29, 531–556 (2008).
Ropers, D. et al. Visualization of coronary artery anomalies and their anatomic course by contrast-enhanced electron beam tomography and three-dimensional reconstruction. Am. J. Cardiol. 87, 193–197 (2001).
Deibler, A. R., Kuzo, R. S., Vohringer, M. et al. Imaging of congenital anomalies with multislice computed tomography. Mayo Clin. Proc. 79, 1017–1023 (2004).
Schmid, M. et al. Visualization of coronary artery anomalies by contrast-enhanced multi-detector row spiral computed tomography. Int. J. Cardiol. 111, 430–435 (2006).
Datta, J. et al. Anomalous coronary arteries in adults: depiction at multi-detector row CT angiography. Radiology 235, 812–818 (2005).
Shi, H., Aschoff, A. J., Brambs, H. J. & Hoffmann, M. H. Multislice CT imaging of anomalous coronary arteries. Eur. Radiol. 14, 2172–2181 (2004).
Morin, R. L., Gerber, T. C. & McCollough, C. H. Radiation dose in computed tomography of the heart. Circulation 107, 917–922 (2003).
Mettler, F. A., Jr, Huda, W., Yoshizumi, T. T. & Mahesh, M. Effective doses in radiology and diagnostic nuclear medicine: a catalog. Radiology 248, 254–263 (2008).
Mollet, N. R. et al. High-resolution spiral computed tomography coronary angiography in patients referred for diagnostic conventional coronary angiography. Circulation 112, 2318–2323 (2005).
Husmann, L. et al. Feasibility of low-dose coronary CT angiography: first experience with prospective ECG-gating. Eur. Heart J. 29, 191–197 (2008).
Herzog, B. A. et al. First head-to-head comparison of effective radiation dose from low-dose 64-slice CT with prospective ECG-triggering versus invasive coronary angiography. Heart 95, 1656–1661 (2009).
Gosling, O. et al. Cardiac CT: are we underestimating the dose? A radiation dose study utilizing the 2007 ICRP tissue weighting factors and a cardiac specific scan volume. Clin. Radiol. 65, 1013–1017 (2010).
Morcos, S. K. & Thomsen, H. S. Adverse reactions to iodinated contrast media. Eur. Radiol. 11, 1267–1275 (2001).
Post, J. C. et al. Magnetic resonance angiography of anomalous coronary arteries: a new gold standard for delineating the proximal course? Circulation 92, 3163–3171 (1995).
McConnell, M. V. et al. Identification of anomalous coronary arteries and their anatomic course by magnetic resonance coronary angiography. Circulation 92, 3158–3162 (1995).
Bunce, N. H. et al. Coronary artery anomalies: assessment with free-breathing three-dimensional coronary MR angiography. Radiology 227, 201–208 (2003).
Erez, E., Tam, V. K., Doublin, N. A. & Stakes, J., Anomalous coronary artery with aortic origin and course between the great arteries: improved diagnosis, anatomic findings, and surgical treatment. Ann. Thorac. Surg. 82, 973–977 (2006).
Brothers, J. A. et al. Evaluation of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients. J. Am. Coll. Cardiol. 50, 2078–2082 (2007).
Osaki, M., McCrindle, B. W., Van Arsdell, G. & Dipchand, A. I. 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. Pediatr. Cardiol. 29, 24–30 (2008).
Morucutti, G. et al. Radionuclide evidence for reversible ischemia after percutaneous treatment of anomalous right coronary artery with dynamic compression by great vessels. J. Cardiovasc. Med. (Hagerstown). 9, 1134–1137 (2008).
Cohenpour, M. et al. Anomalous origin of left main coronary artery: the value of myocardial scintigraphic and spiral computed tomography scans. Nucl. Med. Rev. Cent. East. Eur. 9, 69–71 (2006).
De Luca, L. et al. Stress-rest myocardial perfusion SPECT for functional assessment of coronary arteries with anomalous origin or course. J. Nucl. Med. 45, 532–536 (2004).
Hernandez-Pampaloni, M., Allada, V., Fishbein, M. C. & Schelbert, H. R. Myocardial perfusion and viability by positron emission tomography in infants and children with coronary abnormalities: correlation with echocardiography, coronary angiography, and histopathology. J. Am. Coll. Cardiol. 41, 618–626 (2003).
Schrale, R. G., Channon, K. M. & Ormerod, O. J. IVUS-guided evaluation and percutaneous intervention in an anomalous left main coronary artery. J. Invasive Cardiol. 19, E195–E198 (2007).
Pijls, N. H. et al. Fractional flow reserve. A useful index to evaluate the influence of an epicardial coronary stenosis on myocardial blood flow. Circulation 92, 3183–3193 (1995).
Pijls, N. H. et al. Measurement of fractional flow reserve to assess the functional severity of coronary-artery stenoses. N. Engl. J. Med. 334, 1703–1708 (1996).
Lim, M. J., Forsberg, M. J., Lee, R. & Kern, M. J. Hemodynamic abnormalities across an anomalous left main coronary artery assessment: evidence for a dynamic ostial obstruction. Catheter. Cardiovasc. Interv. 63, 294–298 (2004).
Tsujita, K. et al. In vivo intravascular ultrasonic assessment of anomalous right coronary artery arising from left coronary sinus. Am. J. Cardiol. 103, 747–751 (2009).
Mirchandani, S. & Phoon, C. K. Management of anomalous coronary arteries from the contralateral sinus. Int. J. Cardiol. 102, 383–389 (2005).
Kaku, B. et al. Clinical features of prognosis of Japanese patients with anomalous origin of the coronary artery. Jpn. Circ. J. 60, 731–741 (1996).
Romp, R. L. Outcome of unroofing procedure for repair of anomalous aortic origin of left or right coronary artery. Ann. Thorac Surg. 76, 589–595 (2003).
Moustafa, S. E., Zehr, K., Mookadam, M., Lorenz, E. C. & Mookadam, F. Anomalous interarterial left coronary artery: an evidence based systematic overview. Int. J. Cardiol. 126, 13–20 (2008).
van Son, J. A. & Haas, G. S. Anomalous origin of left main coronary artery from right sinus of Valsalva: modified surgical treatment to avoid neo-coronary ostial stenosis. Eur. J. Cardiothorac. Surg. 10, 467–469 (1996).
Loop, F. D. et al. Influence of the internal-mammary-artery graft on 10-year survival and other cardiac events. N. Engl. J. Med. 314, 1–6 (1986).
Fedoruk, L. M., Kern, J. A., Peeler, B. B. & Kron, I. L. Anomalous origin of the right coronary artery: right internal thoracic artery to right coronary artery bypass is not the answer. J. Thorac. Cardiovasc. Surg. 133, 456–460 (2007).
Tavaf-Motamen, H. et al. Repair of anomalous origin of right coronary artery from the left sinus of Valsalva. Ann. Thorac. Surg. 85, 2135–2136 (2008).
Davies, J. E. et al. Surgical management of anomalous aortic origin of a coronary artery. Ann. Thorac. Surg. 88, 844–847 (2009).
Gulati, R. et al. Surgical management of coronary artery arising from the wrong coronary sinus, using standard and novel approaches. J. Thorac. Cardiovasc. Surg. 134, 1171–1178 (2007).
Rodefeld, M. D., Culbertson, C. B., Rosenfeld, H. M., Hanley, F. L. & Thompson, L. D. Pulmonary artery translocation: a surgical option for complex anomalous coronary artery anatomy. Ann. Thorac. Surg. 72, 2150–2152 (2001).
Alphonso, N. et al. Anomalous coronary artery from the wrong sinus of Valsalva: a physiologic repair strategy. Ann. Thorac. Surg. 83, 1472–1476 (2007).
Hariharan, R., Kacere, R. D. & Angelini, P. Can stent-angioplasty be a valid alternative to surgery when revascularization is indicated for anomalous origination of a coronary artery from the opposite sinus? Tex. Heart Inst. J. 29, 308–313 (2002).
Doorey, A. J. et al. Six-month success of intracoronary stenting for anomalous coronary arteries associated with myocardial ischemia. Am. J. Cardiol. 86, 580–582, (2000).
Taylor, 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. 133, 428–435 (1997).
Lipsett, J., Cohle, S. D., Berry, P. J., Russell, G. & Byard, R. W. Anomalous coronary arteries: a multicenter pediatric autopsy study. Pediatr. Pathol. 14, 287–300 (1994).
Ohnesorge, B. et al. Cardiac imaging by means of electrocardiographically gated multisection spiral CT: initial experience. Radiology 217, 564–571 (2000).
Flohr, T. G. et al. First performance evaluation of a dual-source CT (DSCT) system. Eur. Radiol. 16, 256–268 (2006).
Reimann, A. J. et al. Dual-source computed tomography: advances of improved temporal resolution in coronary plaque imaging. Invest. Radiol. 42, 196–203 (2007).
Feigenbaum, H. in Heart Disease: A Textbook of Cardiovascular Medicine 3rd edn Ch. 5 (ed. Braunwald, E. M. D.) 83 (W. B. Saunders Co., Philadelphia, 1988).
LaBounty, T. M. et al. Comparison of cardiac computed tomographic angiography to transesophageal echocardiography for evaluation of patients with native valvular heart disease. Am. J. Cardiol. 104, 1421–1428 (2009).
Schuijf, J. D., Achenbach, S. A., de Feyter, P. J. & Bax, J. J. Current applications and limitations of coronary computed tomography angiography in stable coronary artery disease. Heart 97, 330–337 (2011).
Foo, T. K. et al. Feasibility of integrating high-spatial-resolution 3D breath-hold coronary MR angiography with myocardial perfusion and viability examinations. Radiology 235, 1025–1030 (2005).
C. P. Vega, University of California, Irvine, CA, USA is the author of and is solely responsible for the content of the learning objectives, questions, and answers of the Medscape, LLC-accredited continuing medical education activity associated with this article.
The authors declare no competing financial interests.
About this article
Cite this article
Lim, J., Beale, A. & Ramcharitar, S. Anomalous origination of a coronary artery from the opposite sinus. Nat Rev Cardiol 8, 706–719 (2011). https://doi.org/10.1038/nrcardio.2011.147
Case Reports in Cardiology (2019)
Diagnostic Accuracy of Noncontrast Self-navigated Free-Breathing MR Angiography Versus CT Angiography: A Prospective Study in Pediatric Patients with Suspected Anomalous Coronary Arteries
Academic Radiology (2019)
International Journal of Athletic Therapy and Training (2019)
JAMA Cardiology (2019)
Coronary Computed Tomography Angiography-Derived Fractional Flow Reserve in Patients with Anomalous Origin of the Right Coronary Artery from the Left Coronary Sinus
Korean Journal of Radiology (2019)