{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T01:47:45Z","timestamp":1782438465410,"version":"3.54.5"},"reference-count":54,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2026,6,25]],"date-time":"2026-06-25T00:00:00Z","timestamp":1782345600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Coronary artery anomalies (CAAs) are a rare but clinically significant group of congenital abnormalities that are associated with myocardial ischaemia, malignant arrhythmias and sudden cardiac death, particularly in young people and athletes. Despite increasing recognition of these conditions and advances in cardiovascular imaging, there are still significant challenges regarding their classification, risk stratification and management, particularly with respect to surgical indications. This review provides a comprehensive overview of the current evidence on the classification, pathophysiology, diagnosis and management of CAAs, with a particular focus on surgical decision-making and contemporary guideline recommendations. A systematic literature search was conducted up to February 2025 using PubMed and Google Scholar. Priority was given to international guidelines, consensus statements, systematic reviews, meta-analyses and large observational studies. CAAs encompass a broad spectrum of anatomical variants and clinical presentations. Among these, anomalies of coronary origin are the most extensively studied. Surgical management is well established for coronary arteries arising from the pulmonary artery, particularly for anomalous left coronary artery from the pulmonary artery (ALCAPA). Substantial advances have also been made in the diagnosis, risk stratification, and treatment of anomalous aortic origin of a coronary artery (AAOCA), which has become a major focus of contemporary guideline recommendations. For patients with AAOCA, surgical correction, including unroofing, coronary reimplantation or coronary artery bypass grafting, is recommended for individuals with symptoms and\/or high-risk anatomical features. In contrast, the diagnosis and management of myocardial bridging, coronary artery fistulas, and coronary artery ectasia remain controversial, with considerable variability in the indications for medical, percutaneous, and surgical treatment. The management of CAAs is an evolving field. While there is consensus for a limited number of anomalies, most cases still require individualised decision-making. It is essential to develop standardised diagnostic frameworks, improved risk stratification tools and outcome-based management criteria. A multidisciplinary, evidence-based approach involving cardiologists, cardiac imagers, interventional cardiologists and cardiac surgeons is crucial in order to optimise patient outcomes and reduce the risk of adverse cardiovascular events, including sudden cardiac death.<\/jats:p>","DOI":"10.3390\/jcm15134959","type":"journal-article","created":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T00:49:23Z","timestamp":1782434963000},"page":"4959","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Coronary Artery Anomalies Revisited: Description of the Types, Pathophysiology and Treatment Options Based on Latest Guidelines"],"prefix":"10.3390","volume":"15","author":[{"given":"Alexandre","family":"Genoud","sequence":"first","affiliation":[{"name":"Faculty of Medicine, University of Geneva, 1205 Geneva, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ines","family":"Portugal","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, University of Geneva, 1205 Geneva, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7157-3880","authenticated-orcid":false,"given":"Nicolas","family":"Murith","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, University of Geneva, 1205 Geneva, Switzerland"},{"name":"Cardiovascular Surgery Division, Department of Surgery, University Hospitals of Geneva, 1205 Geneva, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Francois","family":"Deux","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, University of Geneva, 1205 Geneva, Switzerland"},{"name":"Radiology Division, Department of Diagnostic, University Hospitals of Geneva, 1205 Geneva, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7933-5767","authenticated-orcid":false,"given":"Tornike","family":"Sologashvili","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, University of Geneva, 1205 Geneva, Switzerland"},{"name":"Cardiovascular Surgery Division, Department of Surgery, University Hospitals of Geneva, 1205 Geneva, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Huber","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, University of Geneva, 1205 Geneva, Switzerland"},{"name":"Cardiovascular Surgery Division, Department of Surgery, University Hospitals of Geneva, 1205 Geneva, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1143-347X","authenticated-orcid":false,"given":"Mustafa","family":"Cikirikcioglu","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, University of Geneva, 1205 Geneva, Switzerland"},{"name":"Cardiovascular Surgery Division, Department of Surgery, University Hospitals of Geneva, 1205 Geneva, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,6,25]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1296","DOI":"10.1161\/CIRCULATIONAHA.106.618082","article-title":"Coronary artery anomalies: An entity in search of an identity","volume":"115","author":"Angelini","year":"2007","journal-title":"Circulation"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.1016\/j.jaccas.2022.06.014","article-title":"Case Series of Coronary Artery Anomalies in Athletes: Challenges in Clinical Management and Sports Eligibility","volume":"4","author":"Tso","year":"2022","journal-title":"JACC Case Rep."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"2034","DOI":"10.1016\/j.jacc.2023.08.012","article-title":"Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review","volume":"82","author":"Gaudino","year":"2023","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_4","first-page":"271","article-title":"Coronary artery anomalies--current clinical issues: Definitions, classification, incidence, clinical relevance, and treatment guidelines","volume":"29","author":"Angelini","year":"2002","journal-title":"Tex. Heart Inst. J."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"537","DOI":"10.4329\/wjr.v8.i6.537","article-title":"Coronary artery anomalies overview: The normal and the abnormal","volume":"8","author":"Villa","year":"2016","journal-title":"World J. Radiol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1016\/0002-8703(89)90789-8","article-title":"Normal and anomalous coronary arteries: Definitions and classification","volume":"117","author":"Angelini","year":"1989","journal-title":"Am. Heart J."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"799","DOI":"10.1007\/978-3-031-44087-8_47","article-title":"Clinical Presentation and Therapy of Coronary Artery Anomalies","volume":"1441","author":"Haas","year":"2024","journal-title":"Adv. Exp. Med. Biol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"401","DOI":"10.4037\/ajcc2011734","article-title":"Coronary artery dominance","volume":"20","author":"Pelter","year":"2011","journal-title":"Am. J. Crit. Care"},{"key":"ref_9","first-page":"28","article-title":"Coronary artery anomalies in 126,595 patients undergoing coronary arteriography","volume":"21","author":"Yamanaka","year":"1990","journal-title":"Cathet Cardiovasc. Diagn."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"781","DOI":"10.1007\/s00330-007-0821-9","article-title":"Prevalence of anatomical variants and coronary anomalies in 543 consecutive patients studied with 64-slice CT coronary angiography","volume":"18","author":"Cademartiri","year":"2008","journal-title":"Eur. Radiol."},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Ghadri, J.R., Kazakauskaite, E., Braunschweig, S., Burger, I.A., Frank, M., Fiechter, M., Gebhard, C., Fuchs, T.A., Templin, C., and Gaemperli, O. (2014). Congenital coronary anomalies detected by coronary computed tomography compared to invasive coronary angiography. BMC Cardiovasc. Disord., 14.","DOI":"10.1186\/1471-2261-14-81"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1161\/CIRCULATIONAHA.121.055347","article-title":"Coronary Artery Anomalies","volume":"144","author":"Gentile","year":"2021","journal-title":"Circulation"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1177\/000331970505600104","article-title":"Validation of a clinical-significance-based classification of coronary artery anomalies","volume":"56","author":"Rigatelli","year":"2005","journal-title":"Angiology"},{"key":"ref_14","first-page":"453","article-title":"Identifying, characterizing, and classifying congenital anomalies of the coronary arteries","volume":"32","author":"Shriki","year":"2012","journal-title":"Radiogr. Rev. Publ. Radiol. Soc. N. Am. Inc."},{"key":"ref_15","first-page":"553","article-title":"ALCAPA syndrome: Not just a pediatric disease","volume":"29","author":"Nguyen","year":"2009","journal-title":"Radiogr. Rev. Publ. Radiol. Soc. N. Am. Inc."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"3724","DOI":"10.3760\/cma.j.issn.0366-6999.20133224","article-title":"Two congenital coronary abnormalities affecting heart function: Anomalous origin of the left coronary artery from the pulmonary artery and congenital left main coronary artery atresia","volume":"127","author":"Xiao","year":"2014","journal-title":"Chin. Med. J."},{"key":"ref_17","first-page":"390","article-title":"Anomal\u00edas de las arterias coronarias: Una revisi\u00f3n de la literatura y propuesta de una nueva clasificaci\u00f3n","volume":"30","year":"2023","journal-title":"Rev. Colomb. Cardiol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"4561","DOI":"10.1016\/S0735-1097(24)06551-3","article-title":"A rare case of adult type anomalous origin of right coronary artery from pulmonary artery (arcapa)","volume":"83","author":"Wu","year":"2024","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1161\/CIRCIMAGING.113.000278","article-title":"Novel imaging of coronary artery anomalies to assess their prevalence, the causes of clinical symptoms, and the risk of sudden cardiac death","volume":"7","author":"Angelini","year":"2014","journal-title":"Circ. Cardiovasc. Imaging"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1016\/j.radcr.2018.02.009","article-title":"Transseptal course of anomalous left main coronary artery originating from single right coronary orifice presenting as unstable angina","volume":"13","author":"Glushko","year":"2018","journal-title":"Radiol. Case Rep."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1007\/s12410-015-9339-8","article-title":"Coronary Artery Anomalies: A Pictorial Review","volume":"8","author":"McLarry","year":"2015","journal-title":"Curr. Cardiovasc. Imaging Rep."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/0033-0620(83)90019-1","article-title":"Myocardial bridges: A review","volume":"26","author":"Angelini","year":"1983","journal-title":"Prog. Cardiovasc. Dis."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"2196","DOI":"10.1016\/j.jacc.2021.09.859","article-title":"Myocardial Bridging: Diagnosis, Functional Assessment, and Management: JACC State-of-the-Art Review","volume":"78","author":"Sternheim","year":"2021","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"2616","DOI":"10.1161\/01.CIR.0000038420.14867.7A","article-title":"Update on myocardial bridging","volume":"106","author":"Hort","year":"2002","journal-title":"Circulation"},{"key":"ref_25","first-page":"e43132","article-title":"A Comprehensive Review of Myocardial Bridging: Exploring Diagnostic and Treatment Modalities","volume":"15","author":"Evbayekha","year":"2023","journal-title":"Cureus"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"446","DOI":"10.4330\/wjc.v13.i9.446","article-title":"Coronary artery aneurysm: A review","volume":"13","author":"Matta","year":"2021","journal-title":"World J. Cardiol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1136\/hrt.54.4.392","article-title":"Coronary artery ectasia. Its prevalence and clinical significance in 4993 patients","volume":"54","author":"Hartnell","year":"1985","journal-title":"Br. Heart J."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1393","DOI":"10.1016\/j.jcin.2021.02.044","article-title":"Coronary Artery Fistulas: Indications, Techniques, Outcomes, and Complications of Transcatheter Fistula Closure","volume":"14","author":"AlKhouli","year":"2021","journal-title":"JACC Cardiovasc. Interv."},{"key":"ref_29","first-page":"336","article-title":"The Role of Intravascular Ultrasound (IVUS) In Guiding Interventions for Patients with Aberrant Coronary Arteries: A Comprehensive Review","volume":"5","author":"Contreras","year":"2025","journal-title":"Int. J. Med. Sci. Clin. Res. Stud."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1007\/s11886-019-1188-7","article-title":"Imaging Approaches for Coronary Artery Anomalies: Purpose and Techniques","volume":"21","author":"Angelini","year":"2019","journal-title":"Curr. Cardiol. Rep."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1093\/eurheartj\/ehaa554","article-title":"2020 ESC Guidelines for the management of adult congenital heart disease","volume":"42","author":"Baumgartner","year":"2021","journal-title":"Eur. Heart J."},{"key":"ref_32","first-page":"e698\u2013800","article-title":"2018 AHA\/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology\/American Heart Association Task Force on Clinical Practice Guidelines","volume":"139","author":"Stout","year":"2019","journal-title":"Circulation"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.1016\/j.athoracsur.2024.01.016","article-title":"Anomalous Aortic Origin of a Coronary Artery","volume":"117","author":"Kimbiris","year":"2024","journal-title":"Ann. Thorac. Surg."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1297","DOI":"10.1016\/j.athoracsur.2025.01.029","article-title":"Outcomes of 230 Patients Undergoing Surgical Repair of Anomalous Aortic Origin of a Coronary Artery","volume":"119","author":"Mainwaring","year":"2025","journal-title":"Ann. Thorac. Surg."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1053\/j.optechstcvs.2017.07.001","article-title":"Surgical Unroofing for Anomalous Aortic Origin of Coronary Arteries","volume":"21","author":"Schubert","year":"2016","journal-title":"Oper. Tech. Thorac. Cardiovasc. Surg."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1053\/j.optechstcvs.2017.06.001","article-title":"Surgical Treatment of Anomalous Aortic Origin of Coronary Arteries: The Reimplantation Technique and Its Modifications","volume":"21","author":"Carrel","year":"2016","journal-title":"Oper. Tech. Thorac. Cardiovasc. Surg."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1093\/ejcts\/ezz080","article-title":"Surgery for anomalous aortic origin of coronary arteries: A multicentre study from the European Congenital Heart Surgeons Association\u2020","volume":"56","author":"Padalino","year":"2019","journal-title":"Eur. J. Cardio-Thorac. Surg."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1500","DOI":"10.1016\/j.hlc.2023.09.014","article-title":"Coronary Artery Bypass Surgery to Treat Anomalous Origin of Coronary Arteries in Adults: A Systematic Review","volume":"32","author":"Alzghari","year":"2023","journal-title":"Heart Lung Circ."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1016\/j.athoracsur.2020.04.149","article-title":"Early Outcomes of Transconal Repair of Transseptal Anomalous Left Coronary Artery From Right Sinus","volume":"112","author":"Najm","year":"2021","journal-title":"Ann. Thorac. Surg."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"792","DOI":"10.1177\/2150135120939462","article-title":"Pulmonary Root Mobilization and Modified Lecompte Maneuver for Transseptal Course of the Left Main Coronary Artery","volume":"11","author":"Said","year":"2020","journal-title":"World J. Pediatr. Congenit. Heart Surg."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"e027098","DOI":"10.1161\/JAHA.122.027098","article-title":"Therapeutic Management of Anomalous Coronary Arteries Originating From the Opposite Sinus of Valsalva: Current Evidence, Proposed Approach, and the Unknowing","volume":"11","author":"Bigler","year":"2022","journal-title":"J. Am. Heart Assoc."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1053\/j.pcsu.2025.02.006","article-title":"What is the Optimal Surgical Technique for Anomalous Aortic Origin of a Coronary Artery?","volume":"Volume 28","author":"Mery","year":"2025","journal-title":"Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"E065","DOI":"10.1532\/hsf.3421","article-title":"Current Strategies for the Management of Anomalous Origin of Coronary Arteries from the Pulmonary Artery","volume":"24","author":"Beyaz","year":"2021","journal-title":"Heart Surg. Forum."},{"key":"ref_44","doi-asserted-by":"crossref","unstructured":"Blickenstaff, E.A., Smith, S.D., Cetta, F., Connolly, H.M., and Majdalany, D.S. (2023). Anomalous Left Coronary Artery from the Pulmonary Artery: How to Diagnose and Treat. J. Pers. Med., 13.","DOI":"10.3390\/jpm13111561"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"1063","DOI":"10.1016\/j.athoracsur.2020.01.082","article-title":"Anomalous Origin of the Right Coronary Artery From the Pulmonary Artery: A Systematic Review","volume":"110","author":"Guenther","year":"2020","journal-title":"Ann. Thorac. Surg."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"946","DOI":"10.1016\/S0003-4975(02)03633-0","article-title":"Anomalous origin of the left coronary artery from the pulmonary artery: Collective review of surgical therapy","volume":"74","author":"Mavroudis","year":"2002","journal-title":"Ann. Thorac. Surg."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1016\/j.ehj.2018.10.005","article-title":"Congenital atresia of left main coronary artery","volume":"70","author":"Saedi","year":"2018","journal-title":"Egypt Heart J."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1016\/S1010-7940(96)01121-9","article-title":"Left main coronary artery atresia: Literature review and therapeutical considerations","volume":"11","author":"Musiani","year":"1997","journal-title":"Eur. J. Cardio-Thorac. Surg."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1016\/S0002-8703(99)70123-7","article-title":"Augmentation of vessel squeezing at coronary-myocardial bridge by nitroglycerin: Study by quantitative coronary angiography and intravascular ultrasound","volume":"138","author":"Hongo","year":"1999","journal-title":"Am. Heart J."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"e91","DOI":"10.1016\/j.athoracsur.2018.12.035","article-title":"Alternative Approach for the Surgical Treatment of Left Main Coronary Artery Aneurysm","volume":"108","author":"Haine","year":"2019","journal-title":"Ann. Thorac. Surg."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"24","DOI":"10.3389\/fcvm.2017.00024","article-title":"Coronary Artery Aneurysms: A Review of the Epidemiology, Pathophysiology, Diagnosis, and Treatment","volume":"4","author":"Goktekin","year":"2017","journal-title":"Front Cardiovasc. Med."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"1211","DOI":"10.1016\/j.jcin.2018.02.041","article-title":"Management of Coronary Artery Aneurysms","volume":"11","author":"Kawsara","year":"2018","journal-title":"JACC Cardiovasc. Interv."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"940","DOI":"10.1016\/j.hlc.2017.07.014","article-title":"Coronary Artery Fistulae: Anatomy, Diagnosis and Management Strategies","volume":"27","author":"Buccheri","year":"2018","journal-title":"Heart Lung Circ."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"101935","DOI":"10.1016\/j.cpcardiol.2023.101935","article-title":"Coronary Artery Anomalies\u2014State of the Art Review","volume":"48","author":"Lau","year":"2023","journal-title":"Curr. Probl. Cardiol."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/13\/4959\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T01:28:08Z","timestamp":1782437288000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/13\/4959"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,25]]},"references-count":54,"journal-issue":{"issue":"13","published-online":{"date-parts":[[2026,7]]}},"alternative-id":["jcm15134959"],"URL":"https:\/\/doi.org\/10.3390\/jcm15134959","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,25]]}}}