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Congenital and pediatric coronary artery abnormalities

Peter R Koenig, MD, FACC, FASE
Section Editor
John K Triedman, MD
Deputy Editor
Gordon M Saperia, MD, FACC


In early fetal development, the primitive loosely packed myocardium is nourished via sinusoids, which communicate with the heart cavities. Persistence of these sinusoids may lead to coronary artery cameral fistulae. As the myocardium becomes more compact, the sinusoids disappear and give rise to a network of veins, arteries, and capillaries (at approximately 32 days of gestation) that may have connections with other mediastinal vessels. Persistence of these connections may lead to coronary artery fistulae. (See 'Persistent sinusoids' below.)

As the coronary artery network evolves, endothelial buds arise from the base of the truncus arteriosus. It is still unknown if initially there are only two buds, or buds from each potential cusp of the aortic and pulmonary sinuses (six buds) with later involution of all but two buds. These buds later grow and join the coronary artery network that develops from the sinusoids to establish the definitive coronary artery system. Abnormal involution (in the case of six initial buds), bud position, or septation of the truncus arteriosus may lead to the development of an abnormal origin of the coronary arteries.

Given this complex embryology, it is expected that deviations in development may result in various ("abnormal") origins of the coronary arteries from the normal sinuses of Valsalva in the aorta or from the pulmonary artery. Some of these variations may have no clinical importance while others are clearly pathologic. These variations can be associated with underlying congenital heart defects.


In otherwise normal patients, there may be variations in the number, shape, and location of the ostia or origins of the coronary arteries. Most of these variations appear to be of no clinical significance [1], although a high origin of the ostia may reduce diastolic coronary artery blood flow [2].

Separate origins of the right coronary artery (RCA) and its conal branch occur in 50 percent of the population and separate origins of the left circumflex coronary artery (LCx) and left anterior descending artery (LAD) in 1 percent.


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Literature review current through: Sep 2016. | This topic last updated: May 25, 2016.
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