Anomalous origins of coronary arteries

Associate Editor-In-Chief:

Overview
The coronary arteries are usually perpendicular to the aortic wall and they are radially arranged relative to the center of the aorta. The ostia may be rounding, oval, or elliptical, and the position of the ostium does not appear to affect the flow through it.

Anomalous origins of coronary arteries can be classified as Benign Coronary Anomalies and Potentially More Serious Coronary Artery Anomalies or Coronary Arteries Anomalies without Structural Heart Disease and Coronary Arteries Anomalies with Structural Heart Disease.

Benign Coronary Anomalies

 * Separate origin of left anterior descending artery and left circumflex from the left sinus Valsalva
 * Absent left circumflex (with maximally dominant right coronary artery)
 * Origin of left circumflex artery from right coronary artery or right sinus of Valsalva
 * Ectopic origin of right coronary artery or left main trunk from posterior sinus of Valsalva
 * Ectopic coronary origin from the ascending aorta
 * Intercoronary communication
 * Small coronary artery fistula

Potentially More Serious Coronary Artery Anomalies

 * Ectopic coronary origin from the pulmonary artery
 * Left main trunk from pulmonary artery: Bland-White-Garland syndrome
 * Left anterior descending artery from pulmonary artery
 * Right coronary artery from pulmonary artery
 * All coronary arteries from pulmonary artery


 * Ectopic origin of the left coronary artery from the right sinus of Valsalva
 * Left main trunk from right sinus of Valsalva
 * Left anterior descending artery from right sinus of Valsalva
 * Right coronary artery from left sinus of Valsalva

Normal Variations
These anomalies have no any clinical consequences. Separate origin of the conus branch of the right coronary artery is common. The similar anomaly on the left side is ”separate origins of the left anterior descending and left circumflex coronary arteries” occurs in about 1% of people and is more frequent with bicuspid aortic valves.

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

Anomalous Origin of Left Coronary Arterial Branches from Right Sinus of Valsalva
Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

Single Coronary Artery

 * From right coronary sinus
 * Right coronary artery continues as left circumflex artery and Left anterior descending artery.
 * Right coronary artery gives off left main coronary artery
 * Right coronary artery gives off left anterior descending artery and left circumflex artery


 * From left coronary sinus
 * Left main coronary artery gives off left anterior descending artery, left circumflex artery, and right coronary artery
 * Left circumflex artery continues as right coronary artery
 * Left circumflex artery gives off right coronary artery
 * Left anterior descending artery gives off right coronary artery

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

Coronary Atresia
Total absence of the epicardial (extramural) coronary arteries is rare and occurs most often with pulmonary atresia and aortic atresia.

In these congenital anomalies, pressure in the small but hypertrophied right or left ventricle is similar or higher than aortic pressure, and enlarged sinusoids carry blood from the ventricle to be distributed in the distal coronary arteries.

Stenosis or Atresia of a Coronary Ostium

 * Stenosis or atresia of the ostium or first few millimeters of the left main coronary artery is one of the rarest of the congenital coronary artery anomalies.
 * The more distal branches are normal and develop multiple collaterals from the right coronary artery.
 * According to published literature, presentation range is 3 months to 60 years of age. Usual presentation with;
 * Sudden death
 * Congestive heart failure
 * Angina pectoris
 * Myocardial infarction

All Coronary Arteries from Pulmonary Artery

 * Both right and left coronary arteries, or a single coronary artery, may come from the pulmonary trunk.
 * These children do not survive infancy without surgical intervention unless there is a cardiac lesion causing pulmonary hypertension.

Left Anterior Descending Coronary Artery from the Pulmonary Artery
Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

Left Circumflex Coronary Artery from the Pulmonary Artery or Branches
Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

Right Coronary Artery from the Pulmonary Artery

 * Rare, only about 10% of left main coronary artery originated from the pulmonary artery.
 * Almost always found as an incidental autopsy finding of sudden death, and if diagnosed before death it has been associated with:
 * Symptoms of myocardial ischemia
 * Syncope
 * Cardiomyopathy

Additional Readings

 * Moss and Adams' Heart Disease in Infants, Children, and Adolescents Hugh D. Allen, Arthur J. Moss, David J. Driscoll, Forrest H. Adams, Timothy F. Feltes, Robert E. Shaddy, 2007 ISBN 0781786843
 * Braunwald's Heart Disease, Libby P, 8th ed., 2007, ISBN 978-1-41-604105-4
 * Hurst's the Heart, Fuster V, 12th ed. 2008, ISBN 978-0-07-149928-6
 * Willerson JT, Cardiovascular Medicine, 3rd ed., 2007, ISBN 978-1-84628-188-4
 * Mayo Clinic Cardiology, Concise Textbook, 3rd edition, 2007 ISBN 978-0849390579

Video: ALCAPA
