Anomalous coronary artery with aortic origin and course between the great arteries: Improved diagnosis, anatomic findings, and surgical treatment

Background. Anomalous origin of a coronary artery with subsequent coursing between the great vessels is a rare congenital heart defect that may cause myocardial ischemia and sudden death. Because of the fatality rate of this anomaly, many are diagnosed at the postmortem examination, and reports of surgical correction are few. We present our experience with the diagnosis and surgical treatment of this rare coronary anomaly. Methods. Between June 2003 and August 2005, 9 patients (8 males) were diagnosed with anomalous origin of a coronary artery coursing between the great vessels. The mean age was 12 +/- 5.8 years (range, 4 months to 23 years). Three patients had an intramural origin of the coronary artery. One infant had a single coronary artery and was diagnosed during follow-up of other heart defects. The 8 older patients all presented with exertional syncope or chest pain and echocardiography was used for the initial diagnosis. Six patients had coronary artery reimplantation in the correct aortic sinus, 2 patients had unroofing of the intramural coronary segment, and 1 patient had pericardial patch enlargement of his right coronary artery before reimplantation. One patient, the infant, awaits repair at an older age. Results. No postoperative deaths occurred. The mean hospital stay was 5.5 +/- 1.2 days (range, 4 to 8 days). Three patients had transient ST segment changes during the first 24 hours postoperatively. Follow-up was 4 months to 2.5 years. All patients underwent an exercise myocardial perfusion scan 3 months postoperatively without evidence of myocardial ischemia, and all patients remain clinically well. Conclusions. Echocardiography imaging of young patients with exertional syncope or chest pain is reliable for the diagnosis of this coronary anomaly. It is surgically correctable; however, individual coronary anatomy may cause the surgical approach to vary. The postoperative outcome is excellent.