Anomalous aortic origin of a coronary artery (AAOCA) is a rare but potentially life-threatening congenital heart defect, occurring in approximately 0.4–0.8% of the population. While many patients remain asymptomatic, certain variants—particularly anomalous origin of the left coronary artery (AAOLCA) with interarterial or intramural courses—carry an elevated risk of sudden cardiac arrest (SCA), especially during exertion. Primary care providers play a critical role in the early identification and coordination of care for patients with AAOCA. Although routine electrocardiograms are typically normal, exertional chest pain or syncope should prompt referral to pediatric cardiology. Echocardiography is often the first-line diagnostic tool, but further imaging with cardiac computed tomography (CCT) and/or magnetic resonance imaging (MRI) is essential for confirming the diagnosis and risk-stratification. Identifiable high-risk anatomical features include a slit-like ostium, intramural course, and acute angle of take-off. Exercise stress testing is used for risk stratification but has low sensitivity and is recommended to be used with adjunctive testing such as stress perfusion imaging and occasionally cardiac catheterization. Surgical repair is indicated in patients with AAOLCA, symptomatic individuals, or those with demonstrable ischemia. Asymptomatic patients with AAORCA and low-risk features may be followed conservatively. Regardless of surgical status, all patients require lifelong cardiology follow-up, periodic imaging, and individualized sports clearance. Additionally, first-degree relatives may warrant screening due to potential familial clustering. AAOCA impacts quality of life, with patients and families often experiencing emotional distress due to lifestyle restrictions. Primary care providers should monitor for psychosocial concerns and coordinate mental health support as needed. This review aims to equip primary care clinicians with practical knowledge to ensure timely referral, appropriate counseling, and long-term support for children with AAOCA.
| Published in | American Journal of Pediatrics (Volume 11, Issue 4) |
| DOI | 10.11648/j.ajp.20251104.17 |
| Page(s) | 244-252 |
| Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
| Copyright |
Copyright © The Author(s), 2025. Published by Science Publishing Group |
Pediatrics, Anomalous Coronary Artery, Congenital Heart Disease
| [1] | Brothers JA, Frommelt MA, Jaquiss RDB, Myerburg RJ, Fraser CD, Tweddell JS. Expert consensus guidelines: Anomalous aortic origin of a coronary artery. Journal of Thoracic and Cardiovascular Surgery. The American Association for Thoracic Surgery; 2017; 153: 1440–57. |
| [2] | Molossi S, Doan T, Sachdeva S. Anomalous Coronary Arteries: A State-of-the-Art Approach. Cardiol Clin. W. B. Saunders; 2023. p. 51–69. |
| [3] | Angelini P. Coronary artery anomalies: An entity in search of an identity. Circulation. 2007. p. 1296–305. |
| [4] | Maron BJ, Haas TS, Ahluwalia A, Murphy CJ, Garberich RF. Demographics and Epidemiology of Sudden Deaths in Young Competitive Athletes: From the United States National Registry. American Journal of Medicine. Elsevier Inc.; 2016; 129: 1170–7. |
| [5] | Brothers J, Carter C, McBride M, Spray T, Paridon S. Anomalous left coronary artery origin from the opposite sinus of Valsalva: Evidence of intermittent ischemia. Journal of Thoracic and Cardiovascular Surgery. The American Association for Thoracic Surgery; 2010; 140: e27–9. |
| [6] | Agrawal H, Mery CM, Krishnamurthy R, Molossi S. Anatomic types of anomalous aortic origin of a coronary artery: A pictorial summary. Congenit Heart Dis. Blackwell Publishing Ltd; 2017; 12: 603–6. |
| [7] | Cheezum MK, Ghoshhajra B, Bittencourt MS, Hulten EA, Bhatt A, Mousavi N, et al. Anomalous origin of the coronary artery arising from the opposite sinus : prevalence and outcomes in patients undergoing coronary CTA. 2017; 224–35. |
| [8] | Schiavone M, Gobbi C, Gasperetti A, Zuffi A, Forleo GB. Congenital Coronary Artery Anomalies and Sudden Cardiac Death. Pediatr Cardiol. Springer; 2021. p. 1676–87. |
| [9] | Qasim A, Doan TT, Dan Pham T, Reaves-O’Neal D, Sachdeva S, Mery CM, et al. Is Exercise Stress Testing Useful for Risk Stratification in Anomalous Aortic Origin of a Coronary Artery? Semin Thorac Cardiovasc Surg. W. B. Saunders; 2023; 35: 759–68. |
| [10] | Gentile F, Castiglione V, De Caterina R. Coronary Artery Anomalies. Circulation. Lippincott Williams and Wilkins; 2021. p. 983–96. |
| [11] | Doan TT, Puelz C, Rusin C, Molossi S. Anomalous Aortic Origin of a Coronary Artery in Pediatric Patients. Curr Pediatr Rep. Springer Nature; 2024; 12: 69–80. |
| [12] | Lorber R, Srivastava S, Wilder TJ, Mcintyre S, Decampli WM, Williams WG, et al. Anomalous Aortic Origin of Coronary Arteries in the Young Echocardiographic Evaluation With Surgical Correlation. 2015. |
| [13] | Gaudino M, Di Franco A, Arbustini E, Bacha E, Bates ER, Cameron DE, et al. Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review. J Am Coll Cardiol. Elsevier Inc.; 2023. p. 2034–53. |
| [14] | Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. Elsevier USA; 2019; 73: 1494–563. |
| [15] | Baumgartner H, De Backer J. The ESC clinical practice guidelines for the management of adult congenital heart disease 2020. Eur Heart J. Oxford University Press; 2020. p. 4153–4. |
| [16] | Doan TT, Molossi S, Sachdeva S, Wilkinson JC, Loar RW, Weigand JD, et al. Dobutamine stress cardiac MRI is safe and feasible in pediatric patients with anomalous aortic origin of a coronary artery (AAOCA). Int J Cardiol. Elsevier Ireland Ltd; 2021; 334: 42–8. |
| [17] | Molossi S, Agrawal H, Mery CM, Krishnamurthy R, Masand P, Sexson Tejtel SK, et al. Outcomes in Anomalous Aortic Origin of a Coronary Artery Following a Prospective Standardized Approach. Circ Cardiovasc Interv. Lippincott Williams and Wilkins; 2020; 13: E008445. |
| [18] | Thompson WR. Stress echocardiography in paediatrics: Implications for the evaluation of anomalous aortic origin of the coronary arteries. Cardiol Young. Cambridge University Press; 2015; 25: 1524–30. |
| [19] | Doan TT, Wilkinson JC, Agrawal H, Molossi S, Alam M, Mery CM, et al. Instantaneous Wave-Free Ratio (iFR) Correlates With Fractional Flow Reserve (FFR) Assessment of Coronary Artery Stenoses and Myocardial Bridges in Children. J Invasive Cardiol. 2020; |
| [20] | Molossi S, Sachdeva S. Advice to Young Athletes With Anomalous Aortic Origin of a Coronary Artery With and Without Surgery. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. W. B. Saunders; 2025. p. 83–93. |
| [21] | Kohlsaat K, Gauvreau K, Beroukhim R, Newburger JW, Quinonez L, Nathan M. Trends in surgical management of anomalous aortic origin of the coronary artery over 2 decades. JTCVS Open. Elsevier B. V.; 2023; 16: 757–70. |
| [22] | Mery CM, Beckerman Z. What is the Optimal Surgical Technique for Anomalous Aortic Origin of a Coronary Artery? Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. W. B. Saunders; 2025. p. 94–100. |
| [23] | Brothers JA, Stephens P, Gaynor JW, Lorber R, Vricella LA, Paridon SM. Anomalous Aortic Origin of a Coronary Artery With an Interarterial Course. Should Family Screening Be Routine? J Am Coll Cardiol. 2008; 51: 2062–4. |
| [24] | Agrawal H, Mery CM, Sexson Tejtel SK, Fraser CD, McKenzie ED, Qureshi AM, et al. Familial clustering of cardiac conditions in patients with anomalous aortic origin of a coronary artery and myocardial bridges. Cardiol Young. Cambridge University Press; 2018; 28: 1099–105. |
| [25] | Picazo, B., Pérez-Pomares, J. M. (2016). Human Genetics of Coronary Artery Anomalies. In: Rickert-Sperling, S., Kelly, R., Driscoll, D. (eds) Congenital Heart Diseases: The Broken Heart. Springer, Vienna. |
| [26] | Agrawal H, Mery CM, Sami SA, Qureshi AM, Noel C V., Cutitta K, et al. Decreased Quality of Life in Children With Anomalous Aortic Origin of a Coronary Artery. World J Pediatr Congenit Heart Surg. SAGE Publications Inc.; 2021; 12: 204–10. |
| [27] | Fretay XH du, Boudvillain O, Koutsoukis A, Degrell P, Dupouy P, Aubry P. Catheterization Techniques for Anomalous Aortic Origin of Coronary Arteries. Catheterization and Cardiovascular Interventions. John Wiley and Sons Inc; 2025; 105: 825–37. |
| [28] | Rosato A, Lo Rito M, Anglese S, Ceserani V, Pascaner AF, Secchi F, et al. Assessment of Intramural Segment Compression in Anomalous Coronary Arteries through Patient-Specific Finite Element Modeling. Applied Sciences (Switzerland). Multidisciplinary Digital Publishing Institute (MDPI); 2023; 13. |
| [29] | Razavi A, Sachdeva S, Frommelt PC, LaDisa JF. Patient-Specific Numerical Analysis of Coronary Flow in Children With Intramural Anomalous Aortic Origin of Coronary Arteries. Semin Thorac Cardiovasc Surg. W. B. Saunders; 2021; 33: 155–67. |
| [30] | Puelz C, Rusin CG, Lior D, Sachdeva S, Doan TT, Eilers LF, et al. Fluid-structure interaction simulations for the prediction of fractional flow reserve in pediatric patients with anomalous aortic origin of a coronary artery. 2024; |
| [31] | Lior D, Puelz C, Edwards C, Molossi S, Griffith BE, Birla RK, et al. Semi-Automated Construction of Patient-Specific Aortic Valves from Computed Tomography Images. Ann Biomed Eng. Springer; 2023; 51: 189–99. |
APA Style
Qasim, A., Rosenblum, J. M., Epstein, R., Renno, M. S., Nguyen, Q., et al. (2025). Anomalous Coronary Artery Origins: A Practical Overview for the General Pediatrician. American Journal of Pediatrics, 11(4), 244-252. https://doi.org/10.11648/j.ajp.20251104.17
ACS Style
Qasim, A.; Rosenblum, J. M.; Epstein, R.; Renno, M. S.; Nguyen, Q., et al. Anomalous Coronary Artery Origins: A Practical Overview for the General Pediatrician. Am. J. Pediatr. 2025, 11(4), 244-252. doi: 10.11648/j.ajp.20251104.17
@article{10.11648/j.ajp.20251104.17,
author = {Amna Qasim and Joshua Michael Rosenblum and Rebecca Epstein and Markus Stephan Renno and Quang-Tuyen Nguyen and Ashraf Harahsheh and Seda Tierney and Priyanka Asrani and Rajesh Shenoy and Julie Sue Glickstein and Vladislav Obsekov and Jacob Miller and William Brinson Orr},
title = {Anomalous Coronary Artery Origins: A Practical Overview for the General Pediatrician},
journal = {American Journal of Pediatrics},
volume = {11},
number = {4},
pages = {244-252},
doi = {10.11648/j.ajp.20251104.17},
url = {https://doi.org/10.11648/j.ajp.20251104.17},
eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajp.20251104.17},
abstract = {Anomalous aortic origin of a coronary artery (AAOCA) is a rare but potentially life-threatening congenital heart defect, occurring in approximately 0.4–0.8% of the population. While many patients remain asymptomatic, certain variants—particularly anomalous origin of the left coronary artery (AAOLCA) with interarterial or intramural courses—carry an elevated risk of sudden cardiac arrest (SCA), especially during exertion. Primary care providers play a critical role in the early identification and coordination of care for patients with AAOCA. Although routine electrocardiograms are typically normal, exertional chest pain or syncope should prompt referral to pediatric cardiology. Echocardiography is often the first-line diagnostic tool, but further imaging with cardiac computed tomography (CCT) and/or magnetic resonance imaging (MRI) is essential for confirming the diagnosis and risk-stratification. Identifiable high-risk anatomical features include a slit-like ostium, intramural course, and acute angle of take-off. Exercise stress testing is used for risk stratification but has low sensitivity and is recommended to be used with adjunctive testing such as stress perfusion imaging and occasionally cardiac catheterization. Surgical repair is indicated in patients with AAOLCA, symptomatic individuals, or those with demonstrable ischemia. Asymptomatic patients with AAORCA and low-risk features may be followed conservatively. Regardless of surgical status, all patients require lifelong cardiology follow-up, periodic imaging, and individualized sports clearance. Additionally, first-degree relatives may warrant screening due to potential familial clustering. AAOCA impacts quality of life, with patients and families often experiencing emotional distress due to lifestyle restrictions. Primary care providers should monitor for psychosocial concerns and coordinate mental health support as needed. This review aims to equip primary care clinicians with practical knowledge to ensure timely referral, appropriate counseling, and long-term support for children with AAOCA.},
year = {2025}
}
TY - JOUR T1 - Anomalous Coronary Artery Origins: A Practical Overview for the General Pediatrician AU - Amna Qasim AU - Joshua Michael Rosenblum AU - Rebecca Epstein AU - Markus Stephan Renno AU - Quang-Tuyen Nguyen AU - Ashraf Harahsheh AU - Seda Tierney AU - Priyanka Asrani AU - Rajesh Shenoy AU - Julie Sue Glickstein AU - Vladislav Obsekov AU - Jacob Miller AU - William Brinson Orr Y1 - 2025/12/29 PY - 2025 N1 - https://doi.org/10.11648/j.ajp.20251104.17 DO - 10.11648/j.ajp.20251104.17 T2 - American Journal of Pediatrics JF - American Journal of Pediatrics JO - American Journal of Pediatrics SP - 244 EP - 252 PB - Science Publishing Group SN - 2472-0909 UR - https://doi.org/10.11648/j.ajp.20251104.17 AB - Anomalous aortic origin of a coronary artery (AAOCA) is a rare but potentially life-threatening congenital heart defect, occurring in approximately 0.4–0.8% of the population. While many patients remain asymptomatic, certain variants—particularly anomalous origin of the left coronary artery (AAOLCA) with interarterial or intramural courses—carry an elevated risk of sudden cardiac arrest (SCA), especially during exertion. Primary care providers play a critical role in the early identification and coordination of care for patients with AAOCA. Although routine electrocardiograms are typically normal, exertional chest pain or syncope should prompt referral to pediatric cardiology. Echocardiography is often the first-line diagnostic tool, but further imaging with cardiac computed tomography (CCT) and/or magnetic resonance imaging (MRI) is essential for confirming the diagnosis and risk-stratification. Identifiable high-risk anatomical features include a slit-like ostium, intramural course, and acute angle of take-off. Exercise stress testing is used for risk stratification but has low sensitivity and is recommended to be used with adjunctive testing such as stress perfusion imaging and occasionally cardiac catheterization. Surgical repair is indicated in patients with AAOLCA, symptomatic individuals, or those with demonstrable ischemia. Asymptomatic patients with AAORCA and low-risk features may be followed conservatively. Regardless of surgical status, all patients require lifelong cardiology follow-up, periodic imaging, and individualized sports clearance. Additionally, first-degree relatives may warrant screening due to potential familial clustering. AAOCA impacts quality of life, with patients and families often experiencing emotional distress due to lifestyle restrictions. Primary care providers should monitor for psychosocial concerns and coordinate mental health support as needed. This review aims to equip primary care clinicians with practical knowledge to ensure timely referral, appropriate counseling, and long-term support for children with AAOCA. VL - 11 IS - 4 ER -