Heart & vascular pathology

Ischemic disease

Anomalous coronary artery

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Last staff update: 31 December 2020

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PubMed Search: Anomalous coronary artery [title]

R. Amita, M.D.
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Cite this page: Amita R. Anomalous coronary artery. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/heartanomalouscoronary.html. Accessed September 27th, 2023.
Definition / general
  • A coronary anomaly is defined as any coronary pattern with a feature (number of ostia, proximal course, termination, etc.) "rarely" encountered in the general population
  • Coronary anomalies affect 1% of the general population
  • Necropsies yield an even lower incidence (0.3%)
  • According to the Sudden Death Committee of the American Heart Association, coronary anomalies cause 19% of deaths in athletes (Circulation 1996;94:850)
  • Burke et. al. reported that in 14 to 40 year olds, coronary anomalies are involved in 12% of sports related sudden cardiac deaths versus 1.2% of non sports related deaths (Am Heart J 1991;121:568)
  • Anomaly: right and left coronary arteries both originate from the same sinus of valsalva
    • Normally, the coronary ostia are round to oval in shape but in this anomaly, the coronary artery has an acute takeoff angle that makes the ostium slit like in shape
    • With increased cardiac output (e.g., exercise), the aorta dilates and upon aortic wall stretching, this slit like ostium becomes severely narrowed
  • Anomaly: high takeoff of coronary arteries
    • Normally, the coronary ostia are located within the sinuses of Valsalva which permits maximal opportunity for coronary artery diastolic filling
    • Location of the ostia in the tubular portion of the aorta (i.e., high takeoff position) may be associated with decreased coronary perfusion
Clinical features
  • Signs / symptoms: chest pain, sudden death, cardiomyopathy, syncope, dyspnea, ventricular fibrillation, myocardial infarction
Diagrams / tables

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Classification of coronary anomalies

  • Thallium exercise stress test may be used for diagnosis but is not sufficiently sensitive to show myocardial perfusion defects
Radiology description
  • Coronary angiography and transesophageal echocardiography are useful
  • Contrast enhanced electron beam tomography: offers excellent spatial resolution and identifies most anomalies of coronary vessels but it uses ionizing radiation and potentially nephrotoxic or allergenic contrast agent
  • MRI: avoids radiation and contrast agents and yields excellent images in determining coronary origination, especially in patients with congenital defects
    • Its greatest limitation is in determining the distal coronary course
    • Hence it is less helpful in evaluating fistulas, coronary origination outside the normal sinuses (e.g. from a ventricle or pulmonary artery) and collateral vessels, and visualization of the posterior descending branch
Case reports
  • If diagnosed antemortem, surgery is the treatment of choice in most cases
  • Takeuchi procedure: used to correct the infantile form of anomalous origin of coronaries by creating a communication between the aorta and the left coronary ostium through the pulmonary artery using tubular material (graft) (J Cardiothorac Surg 2008;3:33)
    • Usually, this technique is performed when direct implantation of the anomalous artery into the aorta is difficult due to unfavorable conditions
    • In the adult form, ligation of the origin of the coronary artery at the pulmonary artery is performed in a combined manner so that flow is either restored or persists through a connection with either the internal thoracic artery or a saphenous vein graft from the ascending aorta
Clinical images

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MRI during cardiac systole and end diastole

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Coronary arteries with 3D reconstructions

Gross description
  • Myocardial bridges ("tunneled" epicardial coronary artery)
    • The coronary arteries which normally course over the epicardial surface of the heart may dip into the myocardium to travel for varying lengths and then reappear on the heart surface
    • The muscle overlying the intramyocardial segment of the epicardial coronary artery is termed a "myocardial bridge" and the artery coursing within the myocardium is called a "tunneled" artery
    • Congenital coronary artery aneurysms are found most commonly in the right coronary artery
    • Abnormal flow patterns within the aneurysm may lead to thrombus formation with subsequent vessel occlusion, distal thromboembolization and myocardial infarction
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