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Cardiovasc Res. 2016 Feb 1;109(2):204-16. doi: 10.1093/cvr/cvv251. Epub 2016 Jan 11.

Congenital coronary artery anomalies: a bridge from embryology to anatomy and pathophysiology--a position statement of the development, anatomy, and pathology ESC Working Group.

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Departamento de Biología Animal, Instituto de Investigación Biomédica de Málaga (IBIMA), Facultad de Ciencias, Universidad de Málaga, Campus de Teatinos s/n, Málaga, Spain Andalusian Center for Nanomedicine and Biotechnology (BIONAND), Campanillas (Málaga), Spain
Intercellular Signalling in Cardiovascular Development and Disease Laboratory, Centro Nacional de Investigaciones Cardiovasculares (CNIC), Madrid, Spain.
Department of Experimental Biology, Universidad de Jaén, Jaén, Spain.
Institute of Genetic Medicine, Newcastle University, Centre for Life, Newcastle upon Tyne, UK.
Royal Brompton Hospital, London, UK.
Marie-Lannelongue Hospital-M3C, Paris-Sud University, Le Plessis-Robinson, France.
Aix-Marseille Université, CNRS, IBDM UMR 7288, Marseille, France.
Institute of Physiology, Academy of Sciences of the Czech Republic v.v.i., Prague, Czech Republic First Faculty of Medicine, Institute of Anatomy, Charles University in Prague, Prague 2, Czech Republic.
Department of Cardiovascular Pathology, St. Georges's University of London, London, UK.
Experimental and Clinical Research Center, Max Planck Institut for Clinical Genetics, Berlin, Germany.
Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padova, Italy.
Department of Anatomy, Embryology and Physiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padova, Italy


Congenital coronary artery anomalies are of major significance in clinical cardiology and cardiac surgery due to their association with myocardial ischaemia and sudden death. Such anomalies are detectable by imaging modalities and, according to various definitions, their prevalence ranges from 0.21 to 5.79%. This consensus document from the Development, Anatomy and Pathology Working Group of the European Society of Cardiology aims to provide: (i) a definition of normality that refers to essential anatomical and embryological features of coronary vessels, based on the integrated analysis of studies of normal and abnormal coronary embryogenesis and pathophysiology; (ii) an animal model-based systematic survey of the molecular and cellular mechanisms that regulate coronary blood vessel development; (iii) an organization of the wide spectrum of coronary artery anomalies, according to a comprehensive anatomical and embryological classification scheme; (iv) current knowledge of the pathophysiological mechanisms underlying symptoms and signs of coronary artery anomalies, with diagnostic and therapeutic implications. This document identifies the mosaic-like embryonic development of the coronary vascular system, as coronary cell types differentiate from multiple cell sources through an intricate network of molecular signals and haemodynamic cues, as the necessary framework for understanding the complex spectrum of coronary artery anomalies observed in human patients.


Anatomy; Congenital heart disease; Coronary arteries; Embryology; Pathology

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