Official reprint from UpToDate®
www.uptodate.com ©2018 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Congenital and pediatric coronary artery abnormalities

Peter R Koenig, MD, FACC, FASE
Section Editor
John K Triedman, MD
Deputy Editor
Gordon M Saperia, MD, FACC


In early fetal development, the primitive loosely packed myocardium is nourished via sinusoids, which communicate with the heart cavities. Persistence of these sinusoids may lead to coronary artery cameral fistulae. As the myocardium becomes more compact, the sinusoids disappear and give rise to a network of veins, arteries, and capillaries (at approximately 32 days of gestation) that may have connections with other mediastinal vessels. Persistence of these connections may lead to coronary artery fistulae. (See 'Persistent sinusoids' below.)

As the coronary artery network evolves, endothelial buds arise from the base of the truncus arteriosus. It is still unknown if initially there are only two buds, or buds from each potential cusp of the aortic and pulmonary sinuses (six buds) with later involution of all but two buds. These buds later grow and join the coronary artery network that develops from the sinusoids to establish the definitive coronary artery system. Abnormal involution (in the case of six initial buds), bud position, or septation of the truncus arteriosus may lead to the development of an abnormal origin of the coronary arteries.

Given this complex embryology, it is expected that deviations in development may result in various ("abnormal") origins of the coronary arteries from the normal sinuses of Valsalva in the aorta or from the pulmonary artery. Some of these variations may have no clinical importance while others are clearly pathologic. These variations can be associated with underlying congenital heart defects.


In otherwise normal patients, there may be variations in the number, shape, and location of the ostia or origins of the coronary arteries. Most of these variations appear to be of no clinical significance [1], although a high origin of the ostia may reduce diastolic coronary artery blood flow [2].

Separate origins of the right coronary artery (RCA) and its conal branch occur in 50 percent of the population and separate origins of the left circumflex coronary artery (LCx) and left anterior descending artery (LAD) in 1 percent.

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Dec 2017. | This topic last updated: May 25, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2018 UpToDate, Inc.
  1. Neufeld HN, Schneeweiss A. Coronary Artery Disease in Infants and Children, Lea and Febiger, Philadelphia 1983.
  3. Cheitlin MD, De Castro CM, McAllister HA. Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva, A not-so-minor congenital anomaly. Circulation 1974; 50:780.
  4. Barth CW 3rd, Roberts WC. Left main coronary artery originating from the right sinus of Valsalva and coursing between the aorta and pulmonary trunk. J Am Coll Cardiol 1986; 7:366.
  5. Lorenz EC, Mookadam F, Mookadam M, et al. A systematic overview of anomalous coronary anatomy and an examination of the association with sudden cardiac death. Rev Cardiovasc Med 2006; 7:205.
  6. Kaushal S, Backer CL, Popescu AR, et al. Intramural coronary length correlates with symptoms in patients with anomalous aortic origin of the coronary artery. Ann Thorac Surg 2011; 92:986.
  7. Taylor AJ, Rogan KM, Virmani R. Sudden cardiac death associated with isolated congenital coronary artery anomalies. J Am Coll Cardiol 1992; 20:640.
  8. Kimbiris D, Iskandrian AS, Segal BL, Bemis CE. Anomalous aortic origin of coronary arteries. Circulation 1978; 58:606.
  9. Davis JA, Cecchin F, Jones TK, Portman MA. Major coronary artery anomalies in a pediatric population: incidence and clinical importance. J Am Coll Cardiol 2001; 37:593.
  10. Brothers J, Gaynor JW, Paridon S, et al. Anomalous aortic origin of a coronary artery with an interarterial course: understanding current management strategies in children and young adults. Pediatr Cardiol 2009; 30:911.
  11. Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 1990; 21:28.
  12. McCaffrey FM, Braden DS, Strong WB. Sudden cardiac death in young athletes. A review. Am J Dis Child 1991; 145:177.
  13. Maron BJ, Carney KP, Lever HM, et al. Relationship of race to sudden cardiac death in competitive athletes with hypertrophic cardiomyopathy. J Am Coll Cardiol 2003; 41:974.
  14. Eckart RE, Scoville SL, Campbell CL, et al. Sudden death in young adults: a 25-year review of autopsies in military recruits. Ann Intern Med 2004; 141:829.
  15. Gersony WM. Management of anomalous coronary artery from the contralateral coronary sinus. J Am Coll Cardiol 2007; 50:2083.
  16. Chaitman BR, Lespérance J, Saltiel J, Bourassa MG. Clinical, angiographic, and hemodynamic findings in patients with anomalous origin of the coronary arteries. Circulation 1976; 53:122.
  17. Frommelt PC, Frommelt MA, Tweddell JS, Jaquiss RD. 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 2003; 42:148.
  18. Graham TP Jr, Driscoll DJ, Gersony WM, et al. Task Force 2: congenital heart disease. J Am Coll Cardiol 2005; 45:1326.
  19. McConnell MV, Ganz P, Selwyn AP, et al. Identification of anomalous coronary arteries and their anatomic course by magnetic resonance coronary angiography. Circulation 1995; 92:3158.
  20. Post JC, van Rossum AC, Bronzwaer JG, et al. Magnetic resonance angiography of anomalous coronary arteries. A new gold standard for delineating the proximal course? Circulation 1995; 92:3163.
  21. Taylor AM, Thorne SA, Rubens MB, et al. Coronary artery imaging in grown up congenital heart disease: complementary role of magnetic resonance and x-ray coronary angiography. Circulation 2000; 101:1670.
  22. Bunce NH, Lorenz CH, Keegan J, et al. Coronary artery anomalies: assessment with free-breathing three-dimensional coronary MR angiography. Radiology 2003; 227:201.
  23. Datta J, White CS, Gilkeson RC, et al. Anomalous coronary arteries in adults: depiction at multi-detector row CT angiography. Radiology 2005; 235:812.
  24. Ropers D, Moshage W, Daniel WG, et al. Visualization of coronary artery anomalies and their anatomic course by contrast-enhanced electron beam tomography and three-dimensional reconstruction. Am J Cardiol 2001; 87:193.
  25. Deibler AR, Kuzo RS, Vöhringer M, et al. Imaging of congenital coronary anomalies with multislice computed tomography. Mayo Clin Proc 2004; 79:1017.
  26. Brothers JA, McBride MG, Seliem MA, 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 2007; 50:2078.
  27. Mainwaring RD, Reddy VM, Reinhartz O, et al. Anomalous aortic origin of a coronary artery: medium-term results after surgical repair in 50 patients. Ann Thorac Surg 2011; 92:691.
  28. Romp RL, Herlong JR, Landolfo CK, et al. Outcome of unroofing procedure for repair of anomalous aortic origin of left or right coronary artery. Ann Thorac Surg 2003; 76:589.
  29. Lopez L, Mercer-Rosa L, Zahn EM, et al. The "hinge-twist" technique for anomalous origin of the left coronary artery. Ann Thorac Surg 2006; 82:e19.
  30. Gulati R, Reddy VM, Culbertson C, et al. Surgical management of coronary artery arising from the wrong coronary sinus, using standard and novel approaches. J Thorac Cardiovasc Surg 2007; 134:1171.
  31. Doorey AJ, Pasquale MJ, Lally JF, et al. Six-month success of intracoronary stenting for anomalous coronary arteries associated with myocardial ischemia. Am J Cardiol 2000; 86:580.
  33. Dabizzi RP, Caprioli G, Aiazzi L, et al. Distribution and anomalies of coronary arteries in tetralogy of fallot. Circulation 1980; 61:95.
  34. Wernovsky G, Sanders SP. Coronary artery anatomy and transposition of the great arteries. Coron Artery Dis 1993; 4:148.
  35. Shaher RM, Puddu GC. Coronary arterial anatomy in complete transposition of the great vessels. Am J Cardiol 1966; 17:355.
  36. Elliott LP, Amplatz K, Edwards JE. Coronary arterial patterns in transposition complexes. Anatomic and angiocardiographic studies. Am J Cardiol 1966; 17:362.
  37. Kirklin JW, Barratt-Boyesn BG. Congenitally corrected transposition of the great arteries. In: Cardiac Surgery, Kirklin JW, Barratt-Boyes BG (Eds), John Wiley & Sons, New York 1993. p.1511.
  38. Vlodaver Z, Neufeld HN, Edwards JE. Coronary Artery Variations in the Normal Heart and in Congenital Heart Disease, Academic Press, San Diego 1975.
  39. Anderson KR, McGoon DC, Lie JT. Surgical significance of the coronary arterial anatomy in truncus arteriosus communis. Am J Cardiol 1978; 41:76.
  40. Shrivastava S, Edwards JE. Coronary arterial origin in persistent truncus arteriosus. Circulation 1977; 55:551.
  42. Wright NL, Baue AE, Baum S, et al. Coronary artery steal due to an anomalous left coronary artery originating from the pulmonary artery. J Thorac Cardiovasc Surg 1970; 59:461.
  43. Bland EF, White PD, Garland J. Congenital anomalies of the coronary arteries: report of an unusual case associated with cardiac hypertrophy. Am Heart J 1933; 8:787.
  44. Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases. Circulation 1968; 38:403.
  46. Roberts WC, Robinowitz M. Anomalous origin of the left anterior descending coronary artery from the pulmonary trunk with origin of the right and left circumflex coronary arteries from the aorta. Am J Cardiol 1984; 54:1381.
  47. Roberts WC. Major anomalies of coronary arterial origin seen in adulthood. Am Heart J 1986; 111:941.
  48. Lerberg DB, Ogden JA, Zuberbuhler JR, Bahnson HT. Anomalous origin of the right coronary artery from the pulmonary artery. Ann Thorac Surg 1979; 27:87.
  49. Hoffman JI. Electrocardiogram of anomalous left coronary artery from the pulmonary artery in infants. Pediatr Cardiol 2013; 34:489.
  50. Srinivasan KG, Gaikwad A, Kannan BR, et al. Congenital coronary artery anomalies: diagnosis with 64 slice multidetector row computed tomography coronary angiography: a single-centre study. J Med Imaging Radiat Oncol 2008; 52:148.
  51. Backer CL, Stout MJ, Zales VR, et al. Anomalous origin of the left coronary artery. A twenty-year review of surgical management. J Thorac Cardiovasc Surg 1992; 103:1049.
  52. Waller BF, Orr CM, Slack JD, et al. Anatomy, histology, and pathology of coronary arteries: a review relevant to new interventional and imaging techniques--Part I. Clin Cardiol 1992; 15:451.
  53. Waller BF, Fry ET, Hermiller JB, et al. Nonatherosclerotic causes of coronary artery narrowing--Part II. Clin Cardiol 1996; 19:587.
  54. Angelini P, Trivellato M, Donis J, Leachman RD. Myocardial bridges: a review. Prog Cardiovasc Dis 1983; 26:75.
  55. Visscher DW, Miles BL, Waller BF. Tunneled ("bridged") left anterior descending coronary artery in a newborn without clinical or morphologic evidence of myocardial ischemia. Cathet Cardiovasc Diagn 1983; 9:493.
  56. Bashour TT, Mansour NN, Lee D. Multiple coronary arterial loops as a cause of myocardial ischemia. Am Heart J 1993; 126:219.
  57. Roberts WC, Glick BN. Congenital hypoplasia of both right and left circumflex coronary arteries. Am J Cardiol 1992; 70:121.
  58. Terhune PE, Buchino JJ, Rees AH. Myocardial infarction associated with supravalvular aortic stenosis. J Pediatr 1985; 106:251.
  59. Moore JW, Buchbinder M. Successful coronary stenting in a 4-year-old child. Cathet Cardiovasc Diagn 1998; 44:202.
  60. Stefanadis C, Toutouzas K, Tsiamis E, et al. Stents covered by an autologous arterial graft in porcine coronary arteries: feasibility, vascular injury and effect on neointimal hyperplasia. Cardiovasc Res 1999; 41:433.
  61. Syed M, Lesch M. Coronary artery aneurysm: a review. Prog Cardiovasc Dis 1997; 40:77.
  62. Maehara A, Mintz GS, Ahmed JM, et al. An intravascular ultrasound classification of angiographic coronary artery aneurysms. Am J Cardiol 2001; 88:365.
  63. Hadimeri H, Lamm C, Nyberg G. Coronary aneurysms in patients with autosomal dominant polycystic kidney disease. J Am Soc Nephrol 1998; 9:837.
  64. Eriksen UH, Aunsholt NA, Nielsen TT. Enormous right coronary arterial aneurysm in a patient with type IV Ehlers-Danlos syndrome. Int J Cardiol 1992; 35:259.
  65. Newburger JW, Burns JC. Kawasaki disease. Vasc Med 1999; 4:187.
  66. Suzuki H, Daida H, Tanaka M, et al. Giant aneurysm of the left main coronary artery in Takayasu aortitis. Heart 1999; 81:214.
  67. Hijazi ZM, Udelson JE, Snapper H, et al. Physiologic significance of chronic coronary aneurysms in patients with Kawasaki disease. J Am Coll Cardiol 1994; 24:1633.
  68. Glickel SZ, Maggs PR, Ellis FH Jr. Coronary artery aneurysm. Ann Thorac Surg 1978; 25:372.
  69. Heuser RR, Woodfield S, Lopez A. Obliteration of a coronary artery aneurysm with a PTFE-covered stent: endoluminal graft for coronary disease revisited. Catheter Cardiovasc Interv 1999; 46:113.
  70. Spaedy, TJ, Wilensky, RL. Coronary artery fistulas: clinical implications. ACC Current Journal Review 1994; 3:24.
  71. Hoffman JI. Congenital anomalies of the coronary vessels and the aortic root. In: Heart disease in infants, children and adolescents, 5th ed, Emmanoulides GC, Riemenschneider TA, Allen HD, Gutgesell HP (Eds), Williams and Wilkins, Baltimore 1995. p.780.
  72. Liberthson RR, Sagar K, Berkoben JP, et al. Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management. Circulation 1979; 59:849.
  73. Cotton JL. Diagnosis of a left coronary artery to right ventricular fistula with progression to spontaneous closure. J Am Soc Echocardiogr 2000; 13:225.
  74. Vitarelli A, De Curtis G, Conde Y, et al. Assessment of congenital coronary artery fistulas by transesophageal color Doppler echocardiography. Am J Med 2002; 113:127.
  75. Natarajan A, Khokhar AA, Kirk P, et al. Coronary-pulmonary artery fistula: value of 64-MDCT imaging. QJM 2013; 106:91.
  76. Urrutia-S CO, Falaschi G, Ott DA, Cooley DA. Surgical management of 56 patients with congenital coronary artery fistulas. Ann Thorac Surg 1983; 35:300.
  77. Cheung DL, Au WK, Cheung HH, et al. Coronary artery fistulas: long-term results of surgical correction. Ann Thorac Surg 2001; 71:190.
  78. Armsby LR, Keane JF, Sherwood MC, et al. Management of coronary artery fistulae. Patient selection and results of transcatheter closure. J Am Coll Cardiol 2002; 39:1026.
  79. Reidy JF, Anjos RT, Qureshi SA, et al. Transcatheter embolization in the treatment of coronary artery fistulas. J Am Coll Cardiol 1991; 18:187.
  80. Perry SB, Rome J, Keane JF, et al. Transcatheter closure of coronary artery fistulas. J Am Coll Cardiol 1992; 20:205.
  81. Ogoh Y, Akagi T, Abe T, et al. Successful embolization of coronary arteriovenous fistula using an interlocking detachable coil. Pediatr Cardiol 1997; 18:152.
  82. Hakim F, Madani A, Goussous Y, et al. Transcatheter closure of a large coronary arteriovenous fistula using the new Amplatzer Duct Occluder. Cathet Cardiovasc Diagn 1998; 45:155.
  83. Wilson W, Taubert KA, Gewitz M, et al. Prevention of Infective Endocarditis. Guidelines From the American Heart Association. A Guideline From the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation 2007; 115 published online April 19, 2007. www.circ.ahajournals.org/cgi/reprint/CIRCULATIONAHA.106.183095v1 (Accessed on May 04, 2007).
  84. Hill SL, Hijazi ZM, Hellenbrand WE, Cheatham JP. Evaluation of the AMPLATZER vascular plug for embolization of peripheral vascular malformations associated with congenital heart disease. Catheter Cardiovasc Interv 2006; 67:113.
  85. Kharouf R, Cao QL, Hijazi ZM. Transcatheter closure of coronary artery fistula complicated by myocardial infarction. J Invasive Cardiol 2007; 19:E146.
  86. Garcia JA, Zellers TM, Weinstein EM, Mahony L. Usefulness of Doppler echocardiography in diagnosing right ventricular coronary arterial communications in patients with pulmonary atresia and intact ventricular septum and comparison with angiography. Am J Cardiol 1998; 81:103.
  87. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 2008; 118:e714.
  88. Feltes TF, Bacha E, Beekman RH 3rd, et al. Indications for cardiac catheterization and intervention in pediatric cardiac disease: a scientific statement from the American Heart Association. Circulation 2011; 123:2607.