Left Main Coronary Artery Atresia

HomeRadiologyVol. 309, No. 3 PreviousNext Reviews and CommentaryFree AccessImages in RadiologyLeft Main Coronary Artery AtresiaLeizhi Ku, Xiaojing Ma Leizhi Ku, Xiaojing Ma Author AffiliationsFrom the Departments of Radiology (L.K.) and Echocardiography (X.M.), Wuhan Asia Heart Hospital, No 753 Jinghan Rd, Hankou District, Wuhan, China 430022.Address correspondence to X.M. (email: [email protected]).Leizhi KuXiaojing Ma Published Online:Dec 19 2023https://doi.org/10.1148/radiol.231958MoreSectionsPDF ToolsAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookXLinked In Supplemental material is available for this article.A 67-year-old woman presented to the hospital with chest tightness and pain for 2 years. Her electrocardiogram was unremarkable. Transthoracic echocardiography showed preserved biventricular systolic function, with no regional wall motion abnormalities. Coronary CT angiography (CCTA) demonstrated congenital atresia of the left main coronary artery (LMCA) and stenosis of the mid left anterior descending branch and proximal left circumflex artery (Figure). Three important arterial collateral pathways were observed between the right and left coronary systems: (a) Vieussens arterial ring (Figure), (b) Kugel (retroaortic) anastomotic ring (Figure), and (c) an unnamed collateral pathway connecting the distal right coronary artery to the left circumflex artery (Figure, Movies 1–3). Coronary angiography helped confirm the LMCA atresia with stenosis of the middle anterior descending branch and proximal left circumflex artery (Movies 1, 2). Revascularization was not attempted, as there was no evidence of myocardial ischemia. The patient is currently stable and is being followed up regularly in the outpatient clinic.Images in a 67-year-old woman with left main coronary artery (LMCA) atresia and multiple collateral vessels. (A) Image from coronary CT angiography (CCTA) shows atresia of the LMCA with stenosis of the mid left anterior descending branch and the proximal left circumflex artery. (B) Image from CCTA shows the presence of Vieussens arterial ring between the conus branch of the right coronary artery with the left anterior descending artery. (C) Volume-rendered CCTA image shows the Vieussens arterial ring. (D) Maximum intensity projection image shows a retroaortic Kugel anastomotic ring (arrow). (E) Volume-rendered CCTA image shows an intercoronary collateral vessel communication between the branch of the distal right coronary artery and the distal left circumflex artery. (F) Coronary angiogram confirms LMCA with three intercoronary collateral vessels. AAO = ascending aorta, LA = left atrium, LAD = left anterior descending artery, LCX = left circumflex artery, LM = left main coronary artery, LV = left ventricle, RA = right atrium, RCA = right coronary artery, RV = right ventricle.Download as PowerPoint Movie 1: Coronary angiography confirms that the atresia of the left main coronary artery with stenosis of middle anterior descending branch.Download Original Video (80.6 MB) Movie 2: Coronary angiography confirms that the atresia of the left main coronary artery with stenosis of the proximal left circumflex artery.Download Original Video (72.1 MB) Movie 3: Coronary angiography shows three intercoronary collaterals between the right and left coronary systems, the first large collateral vessel is Vieussens′ arterial ring, it is an anastomotic communication between the conus branch of the RCA and the LAD; the second collateral vessel is Kugel (retroaortic) anastomotic ring, it continues posteriorly behind the aortic root and connects the proximal RCA to the proximal LCX; and the third collateral vessel is unnamed collateral pathway connecting the the distal RCA to the LCX.Download Original Video (48.2 MB)Congenital atresia of the LMCA is rare, with only approximately 60 published cases in the past 70 years (1). In this disorder, there is no left coronary ostium, the proximal LMCA ends blindly, and blood flows from the right coronary artery to the left coronary arterial system via collateral vessels (2). Three major collateral pathways include the Vieussens arterial ring, Kugel anastomic artery, and retroconal anastomotic ring (3). CCTA can complement traditional coronary angiography by providing highly accurate images of coronary arteries and other structural malformations of the heart.Disclosures of conflicts of interest: L.K. No relevant relationships. X.M. No relevant relationships.This work is funded by a 2021 General Project Health and Family Planning Commission of Wuhan Municipality scientific research project (WX21D46) and the Wuhan Science and Technology Project (2019020701011422).References1. Yassin AS, Dayco J, Kottam A. Left Main Coronary Artery Atresia: Diagnostic Images of a Rare Coronary Anomaly. Mayo Clin Proc 2023;98(5):655–656. Crossref, Medline, Google Scholar2. Musiani A, Cernigliaro C, Sansa M, Maselli D, De Gasperis C. Left main coronary artery atresia: literature review and therapeutical considerations. Eur J Cardiothorac Surg 1997;11(3):505–514. Crossref, Medline, Google Scholar3. Doğan N, Dursun A, Özkan H. Vieussens’ arterial ring: a rare coronary variant anatomy. Diagn Interv Radiol 2019;25(2):109–113. Crossref, Medline, Google ScholarArticle HistoryReceived: Aug 1 2023Revision requested: Sept 1 2023Revision received: Sept 5 2023Accepted: Sept 18 2023Published online: Dec 19 2023 FiguresReferencesRelatedDetailsRecommended Articles Anomalous Coronary Arteries That Need Intervention: Review of Pre- and Postoperative Imaging AppearancesRadioGraphics2017Volume: 37Issue: 3pp. 740-757Abnormalities of the Coronary Arteries in Children: Looking beyond the OriginsRadioGraphics2017Volume: 37Issue: 6pp. 1665-1678Coronary Artery Fistulas: Pathophysiology, Imaging Findings, and ManagementRadioGraphics2018Volume: 38Issue: 3pp. 688-703Congenital Coronary Artery–to–Pulmonary Artery Fistula with Anomalous Origin of Right Coronary Artery from Pulmonary Artery: A Case of “Double Trouble”Radiology: Cardiothoracic Imaging2021Volume: 3Issue: 4Spectrum of Coronary Artery Aneurysms: From the Radiologic Pathology ArchivesRadioGraphics2018Volume: 38Issue: 1pp. 11-36See More RSNA Education Exhibits Out on the Road: Revisiting Coronary Artery AnomaliesDigital Posters2022Rarest Of The Rare: Institutional Case-based Presentation Of Unusual Coronary Artery Anomalies Using 3D-Cinematic RenderingsDigital Posters2021Coronary Artery Fistulas: a Pictorial EssayDigital Posters2022 RSNA Case Collection Pulmonary AtresiaRSNA Case Collection2022Anomalous Left Coronary Artery from Pulmonary Artery (ALCAPA)RSNA Case Collection2020Anomalous origin of the right coronary artery from the left sinus of ValsalvaRSNA Case Collection2020 Vol. 309, No. 3 Metrics Altmetric Score PDF download