Patients with anomalous aortic origin of a coronary artery (AAOCA) require imaging
to clarify the multiple potential anatomic sites of obstruction (fixed or dynamic).
Once repaired, the pathway of blood to the myocardium must not encounter: (1) intrinsic
ostial stenosis, (2) obstruction from compression or distortion near the commissure
or the intercoronary pillar, (3) stenosis where the artery exits the aortic wall (due
to an acutely angled “take-off”), (4) compression due to a pathway between the great
vessels, (5) stenosis or compression along an intramural course, or (6) compression
due to an intramuscular (intraseptal/intraconal) course. Detailed anatomic evaluation
of each of these locations allows the surgeon to select an appropriate repair strategy,
and each of these abnormal anatomic features should be “matched” with a particular
surgical correction. We speculate that the most common surgical repair, unroofing
with or without tacking, is often inadequate, as in isolation, it may not allow for
correction with a large orifice from the appropriate sinus, without an interarterial
course. While the evidence base is insufficient to call these recommendations formal
guidelines, these recommendations should serve as a basis for further validity testing,
and ultimate evolution to more granular guidelines on AAOCA management.
Keywords
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Published online: February 02, 2023
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Conflicts of interest: None.
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