Anomalous origins of coronary arteries

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]

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

Potentially More Serious Coronary Artery Anomalies

  • Ectopic origin of the left coronary artery from the right sinus of Valsalva

Coronary Arteries Anomalies without Structural Heart Disease

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. [1] [2]

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

Abnormal Origin of Right or Left Coronary Artery from Inappropriate Sinus

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


Anomalous Origin of Right Coronary Arterial Branches from the Left Sinus of Valsalva

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

Rare Coronary Anomalies

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. [3] [4]

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;

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. [5]

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. [6] [7] [8] [9] [10] [11]
  • 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

References

  1. Baroldi G, Scomazzoni G. Coronary circulation in the normal and the pathologic heart. Washington, DC: Office of the Surgeon General, 1967
  2. Datta J, White CS, Gilkeson RC, Meyer CA, Kansal S, Jani ML, Arildsen RC, Read K Anomalous Coronary Arteries in Adults: Depiction at Multi–Detector Row CT Angiography. Radiology 2005 235: 812-818.
  3. 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 Mar; 11(3):505-14. PMID 9105816
  4. Elian D, Hegesh J, Agranat O, Guetta V, Har-Zahav Y, Rath S, Chouraqui P, Di Segni E. Left main coronary artery atresia: extremely rare coronary anomaly in an asymptomatic adult and an adolescent soccer player. Cardiol Rev. 2003 May-Jun;11(3):160-2. PMID 12705847
  5. Ochoa-Ramirez E, Valdez-Garza HE, Reyes-Gonzalez R, et al. Double anomalous coronary origin from the pulmonary artery: Successful surgical correction in an infant. Tex Heart Inst J 2005;32:348-50.
  6. Coe JY, Radley-Smith R, Yacoub M. Clinical and hemodynamic significance of anomalous origin of the right coronary artery from the pulmonary artery. Thorac Cardiovasc Surg 1982;30:84-7.
  7. Fontana RS, Edwards JE. Congenital Cardiac Disease: A Review of 357 Cases Studied Pathologically. Philadelphia: WB Saunders, 1962.
  8. Ogden JA. Congenital anomalies of the coronary arteries. Am J Cardiol 1970;25:474-79.
  9. Neufeld HN, Schneeweiss A. Coronary Artery Disease in Infants and Children. Philadelphia: Lea & Febiger, 1983.
  10. Roberts WC. Major anomalies of coronary arterial origin seen in adulthood. Am Heart J 1986; 111: 941-63.
  11. Yao CT, Wang JN, Yeh CN, et al. Isolated anomalous origin of right coronary artery from the main pulmonary artery. J Card Surg 2005;20:487-89.

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