Congenital: Anomalous Aortic Origin of Coronary ArteryOutcomes of a protocolized approach for surgical unroofing of intramural anomalous aortic origin of coronary artery in children and adults
Graphical abstract
Section snippets
Materials and Methods
We retrospectively reviewed a total of 148 consecutive patients who underwent surgical unroofing of intramural AAOCA between June 2003 and December 2020 at our institution. Other types of AAOCA, such as intraseptal course or left anterior descending off the right sinus traveling across the right ventricular outflow tract, were not included as part of this study. Research authorization for use of medical records was obtained from all patients, and the Mayo Clinic Institutional Review Board
Results
The median age of the study cohort was 44.4 years (range, 4 months to 83 years) with 40.5% (n = 60) female. The majority were adult patients (80.4%); however, there were 29 patients aged less than 18 years with a median age of 14 years (IQR, 10-16). The AAOCA was an anomalous right coronary artery arising from the left sinus in 130 patients (87.8%), anomalous left coronary artery from the right sinus in 17 patients (11.5%), and anomalous left coronary from the noncoronary sinus in 1 patient (
Discussion
In this large institutional experience with patients having AAOCA, surgical unroofing was performed safely with excellent early outcomes. There was no operative mortality in isolated unroofing and no operative mortality in the pediatric cohort. Overall late survival at 15 years was 94%. Although a minority of patients demonstrated symptoms over follow-up, upon workup none of these patients had ischemia related to AAOCA or the unroofing. All patients were able to resume their activities without
Conclusions
Surgical unroofing of AAOCA can be performed safely even in the setting of concomitant procedures. Management of AAORCA continues to be challenging and requires numerous imaging studies correlated with clinical status and shared decision-making. A protocolized approach has resulted in excellent early results and late survival, with the vast majority being symptom-free.
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