Anomalous aortic origin of a coronary artery: preoperative diagnosis and surgical planning.

BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA), the anomalous coronary artery arises from an inappropriate coronary sinus and travels between the aorta and pulmonary artery. Proper surgical management depends upon correct diagnosis and accurate characterization of the origin and course of the coronary artery. Transthoracic echocardiography (TTE) has been the mainstay for diagnosis, but magnetic resonance imaging (MRI) and computed tomographic angiography (CTA) have been increasingly utilized. In this study, we report the largest series of surgically repaired AAOCA and accuracy of preoperative diagnostic studies. METHODS: A review of 53 consecutive patients (mean age 13.9 years, range 4-65 years) undergoing repair of an AAOCA from 1995 to 2009 was performed. In all, 40 patients were identified with an anomalous right coronary artery (ARCA) from the left sinus of Valsalva, 13 patients had an anomalous left coronary artery (ALCA) arising from the opposite sinus. Symptoms of angina or syncope were present in 58% and 46% of cases with ARCA and ALCA, respectively. RESULTS of preoperative diagnostic testing were compared to actual surgical findings to determine the accuracy of the tests. RESULTS: Lack of an intramural course was observed intraoperatively in 7 cases (5 ARCA and 2 ALCA). Preoperative TTE accurately predicted whether the AAOCA was intramural or extramural in 49 (92.5%) of 53 cases. Magnetic resonance imaging was predictive in 5 (83.3%) of 6 patients and CTA in 11 (64.7%) of 17. Survival was 100%. Complications occurred in 4 (7.5%) of 53 patients (mean follow-up 29 months). Patency was confirmed in 97.7% with TTE, and 23 (95.8%) of 24 patients had a negative postoperative functional study. CONCLUSIONS: Transthoracic echocardiography was found to be very accurate at defining the presence or absence of an intramural course in AAOCA. Both MRI and CTA can provide additional information but may not be as accurate as TTE.