Objective: Anomalous aortic origin of a coronary artery with subsequent coursing between the great vessels is a rare congenital heart defect with increased risk for sudden death. We present our experience with the diagnosis and surgical treatment of this coronary anomaly.
Methods: Since June 2003, 27 patients were diagnosed with anomalous aortic origin of a coronary artery coursing between the great vessels. Age ranged from 4 months to 23 years. The right coronary artery was involved in 20 patients. Six patients had a slit opening and 8 patients had an intramural proximal segment. 15 patients presented with exertional chest pain, 8 experienced syncope and one patient had an acute myocardial infarction.
Diagnosis was made using echocardiography. 18 patients had coronary artery re-implantation in the correct aortic sinus, 5 patients had unroofing of the intramural coronary segment, 3 patients had pericardial patch enlargement of the coronary artery.
Results: There were no postoperative deaths. One patient underwent heart transplantation. 8 patients had transient ST segment changes postoperatively. Hospital stay ranged from 4 to 16 days .
Follow-up ranged from 6 months to 5.5 years. All patients underwent exercise myocardial perfusion scan 3 months postoperatively without evidence of myocardial ischemia and all patients remain clinically well.
Conclusion: Echocardiography imaging of young patients with exertional chest pain or syncope is reliable for the diagnosis of this coronary anomaly. The surgical approach may vary based on individual coronary anatomy. Early and mid- term results are favorable; however, long term coronary anatomy changes should be monitored.