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Anomalous Coronary Origin From Opposite Sinus
Suhny Abbara, MD, FACR, FSCCT; Stephan Achenbach, MD, FESC, FACC, FSCCT; Arzu Canan, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Anomalous aortic origin of coronary artery (AAOCA) originating from opposite sinus
          • Definitions

            • AAOCA arising at or above opposite sinus of Valsalva (AAOCA)
              • AAOCA rarely may originate from noncoronary sinus
            • Anomalous left coronary artery (ALCA)
              • Anomalous origin of left-sided coronary arteries [left main, left anterior descending (LAD) or left circumflex (LCX)] from right sinus of Valsalva or very proximal right coronary artery (RCA)
              • Malignant variant: ALCA courses between ascending aorta and pulmonary artery (PA) (interarterial course)
                • Associated with increased risk for myocardial ischemia or sudden cardiac death (SCD)
              • Benign variant: ALCA that courses outside space between aorta and PA is considered benign
              • Anomalous LCX: Originates from RCA, from common ostium with RCA, or directly from right sinus of Valsalva and courses posterior and inferior to noncoronary cusp toward left side
                • LAD coronary artery originates directly from left sinus of Valsalva
                • No left main (LM) coronary artery segment is present
                • Rarely, anomalous origin of LCX can be from PA
                  • Associated with other major congenital cardiac defects, such as patent ductus arteriosus, aortic coarctation, and subaortic stenosis
            • Anomalous right coronary artery (ARCA)
              • Potentially malignant variant: RCA originates from left sinus of Valsalva and courses between aorta and PA (interarterial)
                • There is debate concerning clinical relevance
                • Risk for SCD assumed low, may be higher if slit-like ostium, acute take-off angle, and intramural course (proximal artery within aortic wall)
              • Benign variants: RCA originates above sinotubular junction superior to right coronary cusp (common) or courses posterior and inferior to aortic root (very rare)
            • Other potentially malignant anomalies include
              • Origin of left coronary artery from PA (ALCAPA, Bland-White-Garland syndrome)
                • Extremely infrequent, usually detected in childhood
                  • ARCAPA: Origin of right coronary artery from PA

          IMAGING

          • General Features

            • Echocardiographic Findings

              • Imaging Recommendations

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Agarwal PP et al: Anomalous Coronary Arteries That Need Intervention: Review of Pre- and Postoperative Imaging Appearances. Radiographics. 37(3):740-757, 2017
                                        2. Cheezum MK et al: Anomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva. J Am Coll Cardiol. 69(12):1592-1608, 2017
                                        3. Camarda J et al: Coronary artery abnormalities and sudden cardiac death. Pediatr Cardiol. 33(3):434-8, 2012
                                        4. Peñalver JM et al: Anomalous aortic origin of coronary arteries from the opposite sinus: a critical appraisal of risk. BMC Cardiovasc Disord. 12:83, 2012
                                        5. Rajiah P et al: Utility of free-breathing, whole-heart, three-dimensional magnetic resonance imaging in the assessment of coronary anatomy for congenital heart disease. Pediatr Cardiol. 32(4):418-25, 2011
                                        6. Young PM et al: Cardiac Imaging: Part 2, Normal, Variant, and Anomalous Configurations of the Coronary Vasculature. AJR. 197:816–826, 2011
                                        7. American College of Cardiology Foundation Task Force on Expert Consensus Documents. et al: ACCF/ACR/AHA/NASCI/SCMR 2010 expert consensus document on cardiovascular magnetic resonance: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents. Circulation. 121(22):2462-508, 2010
                                        8. Clemente A et al: Anomalous origin of the coronary arteries in children: diagnostic role of three-dimensional coronary MR angiography. Clin Imaging. 34(5):337-43, 2010
                                        9. Prakken NH et al: Screening for proximal coronary artery anomalies with 3-dimensional MR coronary angiography. Int J Cardiovasc Imaging. 26(6):701-10, 2010
                                        10. Cheitlin MD et al: Congenital anomalies of coronary arteries: role in the pathogenesis of sudden cardiac death. Herz. 34(4):268-79, 2009
                                        11. Frommelt PC: Congenital coronary artery abnormalities predisposing to sudden cardiac death. Pacing Clin Electrophysiol. 32 Suppl 2:S63-6, 2009
                                        12. Kelle S et al: Coronary MR imaging: lumen and wall. Magn Reson Imaging Clin N Am. 17(1):145-58, 2009
                                        13. Bluemke DA 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(5):586-606, 2008
                                        14. Moustafa SE et al: Anomalous interarterial left coronary artery: an evidence based systematic overview. Int J Cardiol. 126(1):13-20, 2008
                                        15. 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(4):531-56, 2008
                                        16. Angelini P: Coronary artery anomalies: an entity in search of an identity. Circulation. 115(10):1296-305, 2007
                                        17. Dodd JD et al: Congenital anomalies of coronary artery origin in adults: 64-MDCT appearance. AJR Am J Roentgenol. 188(2):W138-46, 2007
                                        18. Jaggers J et al: Surgical therapy for anomalous aortic origin of the coronary arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 122-7, 2005
                                        19. Kim SY et al: Coronary artery anomalies: classification and ECG-gated multi-detector row CT findings with angiographic correlation. Radiographics. 26(2):317-334, 2006
                                        20. Datta J et al: Anomalous coronary arteries in adults: depiction at multi-detector row CT angiography. Radiology. 235(3):812-8, 2005
                                        21. Basso C et al: 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(6):1493-501, 2000
                                        22. McConnell MV et al: Identification of anomalous coronary arteries and their anatomic course by magnetic resonance coronary angiography. Circulation. 92(11):3158-62, 1995
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Anomalous aortic origin of coronary artery (AAOCA) originating from opposite sinus
                                                • Definitions

                                                  • AAOCA arising at or above opposite sinus of Valsalva (AAOCA)
                                                    • AAOCA rarely may originate from noncoronary sinus
                                                  • Anomalous left coronary artery (ALCA)
                                                    • Anomalous origin of left-sided coronary arteries [left main, left anterior descending (LAD) or left circumflex (LCX)] from right sinus of Valsalva or very proximal right coronary artery (RCA)
                                                    • Malignant variant: ALCA courses between ascending aorta and pulmonary artery (PA) (interarterial course)
                                                      • Associated with increased risk for myocardial ischemia or sudden cardiac death (SCD)
                                                    • Benign variant: ALCA that courses outside space between aorta and PA is considered benign
                                                    • Anomalous LCX: Originates from RCA, from common ostium with RCA, or directly from right sinus of Valsalva and courses posterior and inferior to noncoronary cusp toward left side
                                                      • LAD coronary artery originates directly from left sinus of Valsalva
                                                      • No left main (LM) coronary artery segment is present
                                                      • Rarely, anomalous origin of LCX can be from PA
                                                        • Associated with other major congenital cardiac defects, such as patent ductus arteriosus, aortic coarctation, and subaortic stenosis
                                                  • Anomalous right coronary artery (ARCA)
                                                    • Potentially malignant variant: RCA originates from left sinus of Valsalva and courses between aorta and PA (interarterial)
                                                      • There is debate concerning clinical relevance
                                                      • Risk for SCD assumed low, may be higher if slit-like ostium, acute take-off angle, and intramural course (proximal artery within aortic wall)
                                                    • Benign variants: RCA originates above sinotubular junction superior to right coronary cusp (common) or courses posterior and inferior to aortic root (very rare)
                                                  • Other potentially malignant anomalies include
                                                    • Origin of left coronary artery from PA (ALCAPA, Bland-White-Garland syndrome)
                                                      • Extremely infrequent, usually detected in childhood
                                                        • ARCAPA: Origin of right coronary artery from PA

                                                IMAGING

                                                • General Features

                                                  • Echocardiographic Findings

                                                    • Imaging Recommendations

                                                      • CT Findings

                                                        • MR Findings

                                                          • Angiographic Findings

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Agarwal PP et al: Anomalous Coronary Arteries That Need Intervention: Review of Pre- and Postoperative Imaging Appearances. Radiographics. 37(3):740-757, 2017
                                                                              2. Cheezum MK et al: Anomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva. J Am Coll Cardiol. 69(12):1592-1608, 2017
                                                                              3. Camarda J et al: Coronary artery abnormalities and sudden cardiac death. Pediatr Cardiol. 33(3):434-8, 2012
                                                                              4. Peñalver JM et al: Anomalous aortic origin of coronary arteries from the opposite sinus: a critical appraisal of risk. BMC Cardiovasc Disord. 12:83, 2012
                                                                              5. Rajiah P et al: Utility of free-breathing, whole-heart, three-dimensional magnetic resonance imaging in the assessment of coronary anatomy for congenital heart disease. Pediatr Cardiol. 32(4):418-25, 2011
                                                                              6. Young PM et al: Cardiac Imaging: Part 2, Normal, Variant, and Anomalous Configurations of the Coronary Vasculature. AJR. 197:816–826, 2011
                                                                              7. American College of Cardiology Foundation Task Force on Expert Consensus Documents. et al: ACCF/ACR/AHA/NASCI/SCMR 2010 expert consensus document on cardiovascular magnetic resonance: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents. Circulation. 121(22):2462-508, 2010
                                                                              8. Clemente A et al: Anomalous origin of the coronary arteries in children: diagnostic role of three-dimensional coronary MR angiography. Clin Imaging. 34(5):337-43, 2010
                                                                              9. Prakken NH et al: Screening for proximal coronary artery anomalies with 3-dimensional MR coronary angiography. Int J Cardiovasc Imaging. 26(6):701-10, 2010
                                                                              10. Cheitlin MD et al: Congenital anomalies of coronary arteries: role in the pathogenesis of sudden cardiac death. Herz. 34(4):268-79, 2009
                                                                              11. Frommelt PC: Congenital coronary artery abnormalities predisposing to sudden cardiac death. Pacing Clin Electrophysiol. 32 Suppl 2:S63-6, 2009
                                                                              12. Kelle S et al: Coronary MR imaging: lumen and wall. Magn Reson Imaging Clin N Am. 17(1):145-58, 2009
                                                                              13. Bluemke DA 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(5):586-606, 2008
                                                                              14. Moustafa SE et al: Anomalous interarterial left coronary artery: an evidence based systematic overview. Int J Cardiol. 126(1):13-20, 2008
                                                                              15. 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(4):531-56, 2008
                                                                              16. Angelini P: Coronary artery anomalies: an entity in search of an identity. Circulation. 115(10):1296-305, 2007
                                                                              17. Dodd JD et al: Congenital anomalies of coronary artery origin in adults: 64-MDCT appearance. AJR Am J Roentgenol. 188(2):W138-46, 2007
                                                                              18. Jaggers J et al: Surgical therapy for anomalous aortic origin of the coronary arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 122-7, 2005
                                                                              19. Kim SY et al: Coronary artery anomalies: classification and ECG-gated multi-detector row CT findings with angiographic correlation. Radiographics. 26(2):317-334, 2006
                                                                              20. Datta J et al: Anomalous coronary arteries in adults: depiction at multi-detector row CT angiography. Radiology. 235(3):812-8, 2005
                                                                              21. Basso C et al: 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(6):1493-501, 2000
                                                                              22. McConnell MV et al: Identification of anomalous coronary arteries and their anatomic course by magnetic resonance coronary angiography. Circulation. 92(11):3158-62, 1995