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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
Agarwal PP et al: Anomalous Coronary Arteries That Need Intervention: Review of Pre- and Postoperative Imaging Appearances. Radiographics. 37(3):740-757, 2017
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
Camarda J et al: Coronary artery abnormalities and sudden cardiac death. Pediatr Cardiol. 33(3):434-8, 2012
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
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
Young PM et al: Cardiac Imaging: Part 2, Normal, Variant, and Anomalous Configurations of the Coronary Vasculature. AJR. 197:816–826, 2011
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
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
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
Cheitlin MD et al: Congenital anomalies of coronary arteries: role in the pathogenesis of sudden cardiac death. Herz. 34(4):268-79, 2009
Kelle S et al: Coronary MR imaging: lumen and wall. Magn Reson Imaging Clin N Am. 17(1):145-58, 2009
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
Moustafa SE et al: Anomalous interarterial left coronary artery: an evidence based systematic overview. Int J Cardiol. 126(1):13-20, 2008
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
Angelini P: Coronary artery anomalies: an entity in search of an identity. Circulation. 115(10):1296-305, 2007
Dodd JD et al: Congenital anomalies of coronary artery origin in adults: 64-MDCT appearance. AJR Am J Roentgenol. 188(2):W138-46, 2007
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
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
Datta J et al: Anomalous coronary arteries in adults: depiction at multi-detector row CT angiography. Radiology. 235(3):812-8, 2005
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
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
Agarwal PP et al: Anomalous Coronary Arteries That Need Intervention: Review of Pre- and Postoperative Imaging Appearances. Radiographics. 37(3):740-757, 2017
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
Camarda J et al: Coronary artery abnormalities and sudden cardiac death. Pediatr Cardiol. 33(3):434-8, 2012
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
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
Young PM et al: Cardiac Imaging: Part 2, Normal, Variant, and Anomalous Configurations of the Coronary Vasculature. AJR. 197:816–826, 2011
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
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
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
Cheitlin MD et al: Congenital anomalies of coronary arteries: role in the pathogenesis of sudden cardiac death. Herz. 34(4):268-79, 2009
Kelle S et al: Coronary MR imaging: lumen and wall. Magn Reson Imaging Clin N Am. 17(1):145-58, 2009
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
Moustafa SE et al: Anomalous interarterial left coronary artery: an evidence based systematic overview. Int J Cardiol. 126(1):13-20, 2008
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
Angelini P: Coronary artery anomalies: an entity in search of an identity. Circulation. 115(10):1296-305, 2007
Dodd JD et al: Congenital anomalies of coronary artery origin in adults: 64-MDCT appearance. AJR Am J Roentgenol. 188(2):W138-46, 2007
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
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
Datta J et al: Anomalous coronary arteries in adults: depiction at multi-detector row CT angiography. Radiology. 235(3):812-8, 2005
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
McConnell MV et al: Identification of anomalous coronary arteries and their anatomic course by magnetic resonance coronary angiography. Circulation. 92(11):3158-62, 1995
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