Left Main Coronary Artery Atresia: Diagnostic Images of a Rare Coronary Anomaly

Left main coronary artery atresia is very rare coronary anomaly in which the proximal left main trunk ends blindly with a stump; there have been only approximately 60 published cases in the last 70 years. The right coronary artery is the single main coronary artery that partially reconstitutes the left system via multiple small collateral vessels.1Musiani 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: 505-514https://doi.org/10.1016/s1010-7940(96)01121-9Crossref PubMed Scopus (0) Google Scholar Once this condition is diagnosed, surgical reconstruction is recommended for symptomatic patients, with the goal to establish dual blood supply to the myocardium,2Saedi S. Pouraliakbar H.R. Ghaderian H. Saedi T. Congenital atresia of left main coronary artery.Egypt Heart J. 2018; 70: 451-453https://doi.org/10.1016/j.ehj.2018.10.005Crossref PubMed Scopus (7) Google Scholar as these patients are usually symptomatic in the early stages of life with shortness of breath, failure to thrive, syncopal attacks, ventricular arrhythmias, and occasionally they present with sudden cardiac death. Adult older patients develop symptoms at a relatively older age, whereas in pediatric cases, approximately one-third of the patients had associated cardiac malformations.3Serraf A. Baron O. Nottin R. et al.Atrésie ou sténose congénitale de l'ostium coronaire gauche: revascularisation myocardique chez 5 enfants [Atresia or congenital stenosis of the left coronary ostium: myocardial revascularization in 5 children].Arch Mal Coeur Vaiss. 1993; 86: 587-591PubMed Google Scholar The advent of 64-multidetector computed tomography has allowed more awareness and detection of this left main coronary artery atresia.4Nicol E.D. Lyne J. Rubens M.B. Padley S.P. Yen Ho S. Left main coronary atresia: a more commonly identified condition after the advent of 64-slice CT coronary angiography?.J Nucl Cardiol. 2007; 14: 715-718https://doi.org/10.1016/j.nuclcard.2007.05.010Crossref PubMed Scopus (9) Google Scholar A woman in her mid-40s, with no significant medical history, presented with atypical chest pain. She was referred to our institution for computed tomography angiography for evaluation of coronary artery disease. Computed tomography angiography showed a very small-caliber atretic left main coronary artery at the left coronary cusp. The right coronary artery was large and continued as a large posterior descending and a posterior left ventricular artery, thereby reconstituting the left anterior descending artery and left circumflex via collaterals (Figures 1 and 2 and Supplemental Figure 1, available online at http://www.mayoclinicproceedings.org). Coronary angiogram confirmed these findings and revealed no antegrade flow arising from the left sinus of Valsalva, confirming atresia of the left main coronary artery (Supplemental Figure 2, available online at http://www.mayoclinicproceedings.org). The patient was referred for surgical reconstruction, given her symptoms.Figure 2Three-dimensional computed tomography reconstruction showing the reconstructed heart image with atretic left main artery. In here, the left ascending artery rises from the right coronary artery. Left circumflex coronary artery was poorly visualized.View Large Image Figure ViewerDownload Hi-res image Download (PPT) The authors report no competing interests. The authors thank Dr John Dawdy for the procurement and analysis of the images included in the article. Supplemental Figure 2Coronary angiographic image showing an evaluation of the coronary anatomy with patent dominant right coronary artery and collaterals to the left coronary system.View Large Image Figure ViewerDownload Hi-res image Download (PPT)