eMedicine Specialties > Cardiology > Coronary Artery Disease

Isolated Coronary Artery Anomalies: Differential Diagnoses & Workup

Author: Jamshid Shirani, MD, FACC, FAHA, Consulting Staff, Director of Cardiovascular Fellowship Program, Department of Medicine, Division of Cardiology, Geisinger Medical Center
Coauthor(s): Alessandra Brofferio, MD, Fellow, Department of Cardiovascular Medicine, Geisinger Medical Center
Contributor Information and Disclosures

Updated: Mar 13, 2008

Differential Diagnoses

Cardiomyopathy, Dilated
Cardiomyopathy, Hypertrophic
Coronary Artery Atherosclerosis
Patent Ductus Arteriosus
Tetralogy of Fallot
Ventricular Tachycardia

Other Problems to Be Considered

Arrhythmogenic right ventricular dysplasia
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA)
Sudden death

Workup

Imaging Studies

For initial screening purposes, preferred imaging modalities should (1) be noninvasive; (2) be applicable to a wide population, at a reasonable cost, with a minimal level of side effects such as those that could be involved in the use of ionizing radiation; and (3) have reliable diagnostic accuracy.

Noninvasive imaging modalities in patients with coronary artery anomaly are used to either visualize the anomalous vessels or evaluate a heart murmur or symptoms of dyspnea, angina, syncope, or endocarditis. Visualization of anomalous coronary arteries can be achieved by the following noninvasive methods:
 
Transthoracic and transesophageal echocardiography
 
Echocardiography is an attractive screening option in view of its relative simplicity, noninvasiveness, lack of ionizing radiation, relatively low cost, and widespread availability. However, the discriminating power of echocardiography is intrinsically limited (both temporally and geometrically), and few opportunities are available for aligning the echocardiographic imaging planes with the coronary anatomy, which presents curves and phasic movements. Therefore, this technology is less than ideal for firmly diagnosing most types of coronary artery anomalies in adults.   
 
Computed tomographic angiography (CT angio)
 
For studying coronary artery anomalies, CT angio has seen a dramatic rise in interest since the introduction of multidetector computed tomography (MDCT) scanners with 4 detector rows in 1998. The earliest reports on coronary artery anomalies were based on experience with electron-beam computed tomographic (EBCT) scanning, which correlated closely with coronary angiography.

Initial reports concerning the use of MDCT for identifying and characterizing anomalies of coronary origin and course have been quite encouraging, especially considering the benefits of 3-dimensional image reconstruction. Multiple MDCT studies of coronary artery anomalies have already been performed, including studies done to correlate noninvasive findings with that of invasive coronary angiography. Rapid advances in CT angio technology has made this imaging modality a reliable means of defining coronary artery anomalies. However, routine use of CT angio in young patients with suspected coronary anomaly should be discouraged due to exposure to relatively high doses of ionizing radiation. The importance of interpretation skills and proper training in accurate diagnosis of coronary artery anomalies by this modality should be emphasized.
 
Coronary magnetic resonance angiography (MRA)
 
MRA is a noninvasive technique without the disadvantages of CT angio, including ionizing radiation and nephrotoxic contrast agent. As a tomographic imaging technique, MRA allows 3-dimensional reconstruction and omnidirectional visualization of a coronary artery origin and course. In several published series, MRA has been shown to be as accurate as coronary angiography in defining the origin and proximal course of the coronary arteries. However, high resolution definition of the more distal portions of anomalous coronary arteries may be problematic in some patients. Although generally considered safe, MRA is not free of limitations including its inability to be used in patients with claustrophobia or in those with certain metallic implanted devices. Gadolinium-based MR contrast agents have also been implicated in several instances of nephrogenic systemic sclerosis, particularly in patients with advanced kidney disease. 

This modality of imaging is, however, the preferred diagnostic test in younger patients in whom echocardiography has failed to provide adequate definition of the coronary artery anatomy. 
 
Invasive coronary angiography

Definitive diagnosis of coronary artery anomalies at times requires selective arterial angiography via catheterization.

  • During coronary angiography, placing a pulmonary artery flotation (Swan-Ganz) catheter to guide assessment of the course of the anomalous vessels is recommended.
  • Origin of coronary artery from pulmonary trunk may require pulmonary angiography; however, most arteries are visualized during selective arteriography of vessels originating from the aorta.

Other Tests

  • Electrocardiogram (ECG) for evaluation of myocardial ischemia
  • Congenital coronary artery anomalies associated with the origin of the left main or right coronary arteries from the opposite sinus of Valsalva and the type B (ie, between the aorta and pulmonary trunk) course of the anomalous vessel are of particular concern in young competitive athletes. Standard testing with ECG under resting or exercise conditions is unlikely to provide clinical evidence of myocardial ischemia and is not a reliable screening test in athletes. Premonitory cardiac symptoms may occur shortly before sudden death (typically associated with anomalous left main coronary artery), suggesting that a history of exertional syncope or chest pain requires exclusion of this anomaly.
  • Nuclear myocardial perfusion studies using exercise and single-photon emission computed tomography (SPECT) in association with thallium or technetium-based radiolabeled agents can demonstrate ischemia within the myocardial territory supplied by the anomalous coronary artery. Coronary angiography is then recommended to define coronary anatomy.
  • Intracoronary ultrasonography: This invasive study allows evaluation of the mural structure of anomalous coronary arteries in patients in whom invasive coronary angiography fails to clearly demonstrate degree of luminal narrowing or its physiologic significance.

More on Isolated Coronary Artery Anomalies

Overview: Isolated Coronary Artery Anomalies
Differential Diagnoses & Workup: Isolated Coronary Artery Anomalies
Treatment & Medication: Isolated Coronary Artery Anomalies
Follow-up: Isolated Coronary Artery Anomalies
Multimedia: Isolated Coronary Artery Anomalies
References
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References

  1. Angelini P. Normal and anomalous coronary arteries: definitions and classification. Am Heart J. Feb 1989;117(2):418-34. [Medline].

  2. Angelini P. Coronary artery anomalies--current clinical issues: definitions, classification, incidence, clinical relevance, and treatment guidelines. Tex Heart Inst J. 2002;29(4):271-8. [Medline].

  3. Basso C, Maron BJ, Corrado D. 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. May 2000;35(6):1493-501. [Medline].

  4. Becker AE. Congenital coronary arterial anomalies of clinical relevance. Coron Artery Dis. Mar 1995;6(3):187-93. [Medline].

  5. Cieslinski G, Rapprich B, Kober G. Coronary anomalies: incidence and importance. Clin Cardiol. Oct 1993;16(10):711-5. [Medline].

  6. Davis JA, Cecchin F, Jones TK. Major coronary artery anomalies in a pediatric population: incidence and clinical importance. J Am Coll Cardiol. Feb 2001;37(2):593-7. [Medline].

  7. Egred M, Shakespeare CF, Pennell D, Corr L. Magnetic resonance angiography in the assessment of a first reported case of duplicate right coronary artery. Int J Cardiol. May 25 2005;101(2):329-31. [Medline].

  8. Garg N, Tewari S, Kapoor A. Primary congenital anomalies of the coronary arteries: a coronary: arteriographic study. Int J Cardiol. Jun 12 2000;74(1):39-46. [Medline].

  9. Giannoccaro PJ, Sochowski RA, Morton BC. Complementary role of transoesophageal echocardiography to coronary angiography in the assessment of coronary artery anomalies. Br Heart J. Jul 1993;70(1):70-4. [Medline].

  10. Laureti JM, Singh K, Blankenship J. Anomalous coronary arteries: a familial clustering. Clin Cardiol. Oct 2005;28(10):488-90. [Medline].

  11. McConnell MV, Ganz P, Selwyn AP. Identification of anomalous coronary arteries and their anatomic course by magnetic resonance coronary angiography. Circulation. Dec 1 1995;92(11):3158-62. [Medline].

  12. Pucillo AL, Schechter AG, Moggio RA. MR imaging in the definition of coronary artery anomalies. J Comput Assist Tomogr. Mar-Apr 1990;14(2):171-4. [Medline].

  13. Resatoglu AG, Elnur EE, Yener N, Elhassan H, Yener A. Double right coronary artery; fistula and atherosclerosis: rare combination. Jpn J Thorac Cardiovasc Surg. May 2005;53(5):283-5. [Medline].

  14. Roberts WC, Shirani J. The four subtypes of anomalous origin of the left main coronary artery from the right aortic sinus (or from the right coronary artery). Am J Cardiol. Jul 1 1992;70(1):119-21. [Medline].

  15. Ropers D, Moshage W, Daniel WG. Visualization of coronary artery anomalies and their anatomic course by contrast-enhanced electron beam tomography and three-dimensional reconstruction. Am J Cardiol. Jan 15 2001;87(2):193-7. [Medline].

  16. Shirani J, Roberts WC. Solitary coronary ostium in the aorta in the absence of other major congenital cardiovascular anomalies. J Am Coll Cardiol. Jan 1993;21(1):137-43. [Medline].

  17. Shirani J, Roberts WC. Coronary ostial dimple (in the posterior aortic sinus) in the absence of other coronary arterial abnormalities. Am J Cardiol. Jul 1 1993;72(1):118-9. [Medline].

  18. Shirani J, Roberts WC. Origin of the left main coronary artery from the right aortic sinus with retroaortic course of the anomalistically arising artery. Am Heart J. Oct 1992;124(4):1077-8. [Medline].

  19. Spindola-Franco H, Grose R, Solomon N. Dual left anterior descending coronary artery: angiographic description of important variants and surgical implications. Am Heart J. Mar 1983;105(3):445-55. [Medline].

  20. Zeppilli P, dello Russo A, Santini C. In vivo detection of coronary artery anomalies in asymptomatic athletes by echocardiographic screening. Chest. Jul 1998;114(1):89-93. [Medline].

  21. Brofferio A, Shirani J, Chapman J. The pi sign as an indicator of aberrant origin of obtuse marginal coronary artery. Am J Cardiol. Jul 15 2007;100(2):180-1. [Medline].

  22. Bunce NH, Lorenz CH, Keegan J, Lesser J, Reyes EM, Firmin DN. Coronary artery anomalies: assessment with free-breathing three-dimensional coronary MR angiography. Radiology. Apr 2003;227(1):201-8. [Medline].

  23. Casolo G, Del Meglio J, Rega L, Manta R, Margheri M, Villari N. Detection and assessment of coronary artery anomalies by three-dimensional magnetic resonance coronary angiography. Int J Cardiol. Sep 1 2005;103(3):317-22. [Medline].

  24. Post JC, van Rossum AC, Bronzwaer JG, de Cock CC, Hofman MB, Valk J. Magnetic resonance angiography of anomalous coronary arteries. A new gold standard for delineating the proximal course?. Circulation. Dec 1 1995;92(11):3163-71. [Medline].

  25. McConnell MV, Ganz P, Selwyn AP, Li W, Edelman RR, Manning WJ. Identification of anomalous coronary arteries and their anatomic course by magnetic resonance coronary angiography. Circulation. Dec 1 1995;92(11):3158-62. [Medline].

  26. Vliegen HW, Doornbos J, de Roos A, Jukema JW, Bekedam MA, van der Wall EE. Value of fast gradient echo magnetic resonance angiography as an adjunct to coronary arteriography in detecting and confirming the course of clinically significant coronary artery anomalies. Am J Cardiol. Mar 15 1997;79(6):773-6. [Medline].

  27. Stuber M, Botnar RM, Fischer SE, Lamerichs R, Smink J, Harvey P. Preliminary report on in vivo coronary MRA at 3 Tesla in humans. Magn Reson Med. Sep 2002;48(3):425-9. [Medline].

  28. Stuber M, Botnar RM, Danias PG, Sodickson DK, Kissinger KV, Van Cauteren M. Double-oblique free-breathing high resolution three-dimensional coronary magnetic resonance angiography. J Am Coll Cardiol. Aug 1999;34(2):524-31. [Medline].

  29. Memisoglu E, Hobikoglu G, Tepe MS, Norgaz T, Bilsel T. Congenital coronary anomalies in adults: comparison of anatomic course visualization by catheter angiography and electron beam CT. Catheter Cardiovasc Interv. Sep 2005;66(1):34-42. [Medline].

  30. Ropers D, Moshage W, Daniel WG, Jessl J, Gottwik M, Achenbach S. Visualization of coronary artery anomalies and their anatomic course by contrast-enhanced electron beam tomography and three-dimensional reconstruction. Am J Cardiol. Jan 15 2001;87(2):193-7. [Medline].

  31. Yoshimura N, Hamada S, Takamiya M, Kuribayashi S, Kimura K. Coronary artery anomalies with a shunt: evaluation with electron-beam CT. J Comput Assist Tomogr. Sep-Oct 1998;22(5):682-6. [Medline].

  32. Datta J, White CS, Gilkeson RC, Meyer CA, Kansal S, Jani ML. Anomalous coronary arteries in adults: depiction at multi-detector row CT angiography. Radiology. Jun 2005;235(3):812-8. [Medline].

  33. Schmid M, Achenbach S, Ludwig J, Baum U, Anders K, Pohle K. Visualization of coronary artery anomalies by contrast-enhanced multi-detector row spiral computed tomography. Int J Cardiol. Aug 28 2006;111(3):430-5. [Medline].

  34. Schmitt R, Froehner S, Brunn J, Wagner M, Brunner H, Cherevatyy O. Congenital anomalies of the coronary arteries: imaging with contrast-enhanced, multidetector computed tomography. Eur Radiol. Jun 2005;15(6):1110-21. [Medline].

  35. Deibler AR, Kuzo RS, Vöhringer M, Page EE, Safford RE, Patron JN. Imaging of congenital coronary anomalies with multislice computed tomography. Mayo Clin Proc. Aug 2004;79(8):1017-23. [Medline].

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Further Reading

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Keywords

isolated coronary artery anomalies, anomalous origin of epicardial coronary arteries, anomalous course of epicardial coronary arteries, anomalous termination of epicardial coronary arteries, congenital stenosis of the epicardial coronary arteries, atresia of the coronary arteries

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Contributor Information and Disclosures

Author

Jamshid Shirani, MD, FACC, FAHA, Consulting Staff, Director of Cardiovascular Fellowship Program, Department of Medicine, Division of Cardiology, Geisinger Medical Center
Jamshid Shirani, MD, FACC, FAHA is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians, American Federation for Medical Research, American Heart Association, American Society of Echocardiography, and Association of Subspecialty Professors
Disclosure: Nothing to disclose.

Coauthor(s)

Alessandra Brofferio, MD, Fellow, Department of Cardiovascular Medicine, Geisinger Medical Center
Disclosure: Nothing to disclose.

Medical Editor

Park W Willis IV, MD, Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine
Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Steven J Compton, MD, FACC, FACP, Director of Cardiac Electrophysiology, Alaska Heart Institute, Providence and Alaska Regional Hospitals
Steven J Compton, MD, FACC, FACP is a member of the following medical societies: Alaska State Medical Association, American College of Cardiology, American College of Physicians, and Heart Rhythm Society
Disclosure: Nothing to disclose.

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Eric H Yang, MD, Assistant Professor of Medicine, Director of Coronary Care Unit, University of North Carolina at Chapel Hill School of Medicine
Eric H Yang, MD is a member of the following medical societies: Alpha Omega Alpha
Disclosure: Up to Date Royalty Review panel membership; pfizer Honoraria Speaking and teaching

 
 
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