Outcomes of 230 Patients Undergoing Surgical Repair of Anomalous Aortic Origin of a Coronary Artery

Background: Anomalous aortic origin of a coronary artery (AAOCA) is being identified with increasing regularity and may be associated with myocardial ischemia and/or sudden death. There is an on-going controversy regarding which patients should have medical treatment, which should undergo surgical treatment, and what are the optimal surgical techniques to achieve lasting success.

Methods: This was a retrospective review of 230 patients who underwent surgical repair of AAOCA at a single institution. Median age at surgery was 17 years. Preoperative symptoms in 130 patients prompted their referral for surgery, including with sudden death. An additional 32 patients had associated congenital heart defects. Among the cohort, 29% were asymptomatic, 187 had an anomalous right coronary, 43 had an anomalous left coronary, and 192 had an intramural course, whereas 38 did not, including 13 with an intraconal course.

Results: An unroofing procedure was performed in 86 patients, 123 underwent coronary reimplantation, the intraconal left main was repaired in 13, and 8 had other procedures. There were no early or late deaths with a median follow-up of 4 years. Recurrent symptoms and/or ischemia resulted in reoperations in 6 patients (2.6%); of these, 3 had anatomic narrowing of the coronary artery and the other 3 had an undiagnosed myocardial bridge. No reoperations were required in the reimplantation group, and 5 reoperations (5.8%) were in the unroofing cohort.

Conclusions: Surgical repair of AAOCA can be performed with extremely low mortality and low incidence of reoperation. Our current preference is to perform coronary reimplantation because this mitigates some pitfalls of the unroofing procedure.