Congenital and acquired coronary artery disease in children: the challenging patients in paediatric practise

Authors

DOI:

https://doi.org/10.15574/SP.2025.4(148).9096

Keywords:

coronary artery disease, congenital coronary artery pathologies, acquired coronary artery disease

Abstract

Coronary artery disease (CAD) is a rare condition seen in the paediatric population, which makes it difficult to diagnose and manage these patients due to the broad spectrum of its clinical forms that ranges from asymptomatic course to the development of sudden cardiac death syndrome as a result of myocardial ischemia in children. CAD presents as acquired and congenital variants with varying subtypes.

Aim - to focus on aetiology, main clinical features, and early detection methods for CAD, prioritising children and adolescents to make it easier for the paediatric specialists to manage this challenging disease and prevent death.

For this review, a total of 50 articles have been analyzed from PubMed, Uptodate, Researchgate, Google Scholar, etc, which are taken from the past ten years of journals. Congenital CAD presents as defects of origin, course, or termination, and anomalous origin of the left coronary artery from the pulmonary artery, also known as ALCAPA, has the highest prevalence, and it causes 90% deaths within the first year of life. When it comes to acquired CAD, the common aetiology is found to be Kawasaki disease, which is a type of medium vessel vasculitis predominant in children between 6 months and 8 years. Another in line is familial hypercholesterolemia. It is observed that patients aged from 11-23 years, who have a history of familial hypercholesterolemia, 25% children and adolescents were found to have atherosclerotic plaques in the vessels that lead to myocardial ischemia and dysfunction. Accurate diagnosis requires specialized knowledge and skills using cardiac imaging methods. The “gold standard” method in this era is coronary computed tomography angiography, but it is not a routine test; therefore, doctors should educate themselves continuously with the latest updates in order to be able to determine the high-risk groups.

Improved knowledge about coronary artery pathologies can be of great help in diagnosing and managing children and help to prevent life-threatening complications or sudden cardiac death.

The authors declare no conflict of interest.

References

Angelini P. (2014, Jul). Novel imaging of coronary artery anomalies to assess their prevalence, the causes of clinical symptoms, and the risk of sudden cardiac death. Circ Cardiovasc Imaging. 7(4): 747-754. PMID: 25027456. https://doi.org/10.1161/CIRCIMAGING.113.000278; PMid:25027456

Bassareo PP, O'Brien ST, Dunne E, Duignan S, Martino E et al. (2022, Jun 30). Should We Be Screening for Ischaemic Heart Disease Earlier in Childhood? Children (Basel). 9(7): 982. https://doi.org/10.3390/children9070982; PMid:35883966 PMCid:PMC9320497

Bax JJ, Wackers FJ Th, Delgado V. (2022, Jul). Screening for coronary heart disease in patients with diabetes mellitus. Editors: David M Nathan, Patricia A Pellikka. URL: https://www.uptodate.com/contents/screening-for-coronary-heart-disease-in-patients-with-diabetes-mellitus.

Blickenstaff EA, Smith SD, Cetta F, Connolly HM, Majdalany DS. (2023, Oct 31). Anomalous Left Coronary Artery from the Pulmonary Artery: How to Diagnose and Treat. J Pers Med. 13(11): 1561. https://doi.org/10.3390/jpm13111561; PMid:38003878 PMCid:PMC10672344

Clarke SL, Tcheandjieu C, Hilliard AT, Lee KM, Lynch J, Chang KM et al. (2022, Apr). Coronary Artery Disease Risk of Familial Hypercholesterolemia Genetic Variants Independent of Clinically Observed Longitudinal Cholesterol Exposure. Circ Genom Precis Med. 15(2): e003501. Epub 2022 Feb 10. https://doi.org/10.1161/CIRCGEN.121.003501; PMid:35143253 PMCid:PMC10593360

Coeng P, Hijazi ZM. (2024, Nov). Congenital and pediatric coronary artery abnormalities. Editor: John K Triedman, URL: Https://www.uptodate.com/contents/congenital-and-pediatric-coronary-artery-abnormalities.

D'Ascenzi F, Valentini F, Pistoresi S, Frascaro F, Piu P, Cavigli L et al. (2022, Jul). Causes of sudden cardiac death in young athletes and non-athletes: systematic review and meta-analysis: Sudden cardiac death in the young. Trends Cardiovasc Med. 32(5): 299-308. Epub 2021 Jun 22. https://doi.org/10.1016/j.tcm.2021.06.001; PMid:34166791

De Ferranti SD. (2024, Apr). Familial hypercholesterolemia in children. Editors: David R Fulton. URL: Https://Www.Uptodate.Com/Contents/Familial-Hypercholesterolemia-In-Children

De Ferranti SD, Steinberger J, Ameduri R, Baker A, Gooding H, Kelly AS et al. (2019, Mar 26). Cardiovascular Risk Reduction in High-Risk Pediatric Patients: A Scientific Statement From the American Heart Association. Circulation. 139(13): e603-e634. PMID: 30798614. https://doi.org/10.1161/CIR.0000000000000618

Dudnyk VM, Zborovska OO, Demianyshyna VV, Popov VP. (2022). Pathology of coronary arteries in children and its clinical significance. Bulletin of Vinnytsia National Medical University. 26(4). 663-669. https://doi.org/10.31393/reports-vnmedical-2022-26(4)-25; PMid:32935026

Farnier M, Salignon-Vernay C, Yao H, Chague F, Brunel P, Maza M et al. (2019, Jul-Aug). Prevalence, risk factor burden, and severity of coronary artery disease in patients with heterozygous familial hypercholesterolemia hospitalized for an acute myocardial infarction: Data from the French RICO survey. J Clin Lipidol. 13(4): 601-607. Epub 2019 Jun 21. https://doi.org/10.1016/j.jacl.2019.06.005; PMid:31324593

Feng J, Zhao J, Li J, Sun Z, Li Q. (2023, Mar 10). Classification, diagnosis and clinical strategy of congenital coronary artery disease in children. Front Pediatr. 11: 1132522. https://doi.org/10.3389/fped.2023.1132522; PMid:36969282 PMCid:PMC10036911

Franciosi S, Abrams DJ, Ingles J, Sanatani S. (2022, Feb 10). Sudden Cardiac Arrest in the Paediatric Population. CJC Pediatr Congenit Heart Dis. 1(2): 45-59. https://doi.org/10.1016/j.cjcpc.2022.02.001; PMid:37969243 PMCid:PMC10642157

Gao Y, Zhang Q, Sun Y, Du J. (2022, Jul 5). Congenital Anomalous Origin of Coronary Artery Disease in Children With Syncope: A Case Series. Front Pediatr. 10: 879753. https://doi.org/10.3389/fped.2022.879753; PMid:35865709 PMCid:PMC9294363

Gentile F, Castiglione V, De Caterina R. (2021, Sep 21). Coronary Artery Anomalies. Circulation. 144(12): 983-996. Epub 2021 Sep 20. https://doi.org/10.1161/CIRCULATIONAHA.121.055347; PMid:34543069

Geggel RL, Endom EE. (2024, Apr). Causes of nontraumatic chest pain in children and adolescents. Editors: David R Fulton, Gregory Redding, Jan E Drutz, Mark I Neuman. URL: https://www.uptodate.com/contents/causes-of-nontraumatic-chest-pain-in-children-and-adolescents.

Geggel RL, Endom EE. (2024, May). Nontraumatic chest pain in children and adolescents: Approach and initial management. Editors: David R Fulton, Gregory Redding, Mark I Neuman. URL: https://www.uptodate.com/contents/nontraumatic-chest-pain-in-children-and-adolescents-approach-and-initial-management.

Ghadri JR, Kazakauskaite E, Braunschweig S, Burger IA, Frank M, Fiechter M et al. (2014, Jul 8). Congenital coronary anomalies detected by coronary computed tomography compared to invasive coronary angiography. BMC Cardiovasc Disord. 14: 81. https://doi.org/10.1186/1471-2261-14-81; PMid:25004927 PMCid:PMC4118645

Kopecky SL, Halkar MG. (2023, Jan). Screening for coronary heart disease. Editors: Joann G Elmlore, Patricia A Pellikka. URL: https://www.uptodate.com/contents/screening-for-coronary-heart-disease.

Koppel CJ, Jongbloed MRM, Kiès P, Hazekamp MG, Mertens BJA et al. (2020, May 1). Coronary anomalies in tetralogy of Fallot - A meta-analysis. Int J Cardiol. 306: 78-85. Epub 2020 Feb 17. https://doi.org/10.1016/j.ijcard.2020.02.037; PMid:32156463

McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M et al. (2017, Apr 25). Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation. 135(17): e927-e999. Epub 2017 Mar 29. doi: 10.1161/CIR.0000000000000484. Erratum in: Circulation. 2019 Jul 30; 140(5): e181-e184. PMID: 28356445. https://doi.org/10.1161/CIR.0000000000000703

Newburger JW, de Ferranti SD, Fulton DR. (2024, Nov). Cardiovascular sequelae of Kawasaki disease: Clinical features and evaluation. Editor: John K Triedman. URL: https://www.uptodate.com/contents/cardiovascular-sequelae-of-kawasaki-disease-clinical-features-and-evaluation.

Rajasekaran K, Duraiyarasan S, Adefuye M, Manjunatha N, Ganduri V. (2022, Aug 24). Kawasaki Disease and Coronary Artery Involvement: A Narrative Review. Cureus. 14(8): e28358. PMID: 36185934; PMCID: PMC9514671. https://doi.org/10.7759/cureus.28358

Salerno JC. (2024, Jul). Causes of syncope in children and adolescents. Section Editors: George A Woodward, John K Triedman. URL: https://www.uptodate.com/contents/causes-of-syncope-in-children-and-adolescents.

Temel MT, Coşkun ME, Başpınar O, Demiryürek AT. (2017, Sep). Prevalence and characteristics of coronary artery anomalies in children with congenital heart disease diagnosed with coronary angiography. Turk Kardiyol Dern Ars. 45(6): 527-532. https://doi.org/10.5543/tkda.2017.24162; PMid:28902643

Vaezi Z, Amini A. (2022, Sep 26). Familial Hypercholesterolemia. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. PMID: 32310469.

Velasco Forte MN, Valverde I, Prabhu N, Correia T, Narayan SA, Bell A et al. (2019, Feb 25). Visualization of coronary arteries in paediatric patients using whole-heart coronary magnetic resonance angiography: comparison of image-navigation and the standard approach for respiratory motion compensation. J Cardiovasc Magn Reson. 21(1): 13. https://doi.org/10.1186/s12968-019-0525-8; PMid:30798789 PMCid:PMC6388473

Winkel BG, Risgaard B, Sadjadieh G, Bundgaard H, Haunsø S, Tfelt-Hansen J. (2014, Apr). Sudden cardiac death in children (1-18 years): symptoms and causes of death in a nationwide setting. Eur Heart J. 35(13): 868-875. Epub 2013 Dec 15. https://doi.org/10.1093/eurheartj/eht509; PMid:24344190

Yan F, Zhang H, Xiong R, Cheng X, Chen Y, Zhang F. (2020, Nov 20). Effect of Early Intravenous Immunoglobulin Therapy in Kawasaki Disease: A Systematic Review and Meta-Analysis. Front Pediatr. 8: 593435. https://doi.org/10.3389/fped.2020.593435; PMid:33330287 PMCid:PMC7715029

Zeppenfeld K, Tfelt-Hansen J, de Riva M, Winkel BG, Behr ER, Blom NA et al. (2022, Oct 21). 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 43(40): 3997-4126. https://doi.org/10.1093/eurheartj/ehac262; PMid:36017572

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Published

2025-06-15