Anomalous Aortic Origin of a Coronary Artery: Symptoms Do Not Correlate With Intramural Length or Ostial Diameter

BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) is a known cause of sudden death. Our hypothesis was that longer intramural length and smaller ostial diameter correlate with preoperative symptoms. If true, this would assist in the decision for surgical indications. We also assessed the accuracy of preoperative imaging to predict intramural length.

METHODS: Retrospective analysis of patients who underwent AAOCA unroofing from 2006 to 2014. Patients had preoperative computed tomography angiography (CTA) or magnetic resonance imaging (MRI). Intramural length was measured. Intramural lengths and ostial diameters were also measured intraoperatively (operating room [OR]). Symptoms were noted. Intramural lengths and ostial diameters were compared between patients with and without preoperative symptoms. The accuracy of intramural length measured by CTA/MRI versus the length measured in the OR was assessed using a Bland-Altman analysis.

RESULTS: Sixty-six patients underwent surgical repair of AAOCA. Fifty-two (79%) patients were symptomatic and 14 (21%) were asymptomatic. Mean age was 12.4 ± 4.0 years. There was no mortality. There was strong agreement between intramural length measured by CTA/MRI and measured in the OR. There was no significant difference in AAOCA intramural length in the symptomatic (8.6 ± 3.5 mm) and asymptomatic (8.9 ± 2.8 mm, P = .77) patients, which were measured both by CTA/MRI and intraoperatively (symptomatic 7.3 ± 2.5 mm, asymptomatic 6.9 ± 2.8 mm; P = .62). There was also no significant difference in AAOCA ostial diameters between groups (symptomatic = 1.9 ± 0.5 mm, asymptomatic = 1.6 ± 0.5 mm; P = .09).

CONCLUSION: Preoperative CTA/MRI was very accurate in predicting the length of surgical unroofing. There was no demonstrable correlation between preoperative symptoms and intramural AAOCA length or AAOCA ostial diameter.