Diagnosis and treatment of anomalous aortic origin of coronary artery: A twenty-year retrospective study of experience and decision-making in children and young adults

Background: Anomalous aortic origin of a coronary artery, particularly in the presence of inter-arterial course between the great arteries, has been found to be associated with sudden death in young people.

Methods: This study reports a single-center experience in the management of anomalous aortic origin of a coronary artery, with or without inter-arterial course, by focusing specifically on presentation, diagnosis, and patient outcome.

Results: From March 1993 to February 2018, 100 patients (70 males) were diagnosed with anomalous aortic origin of a coronary artery, including 27 left coronary artery from the right sinus, 60 right coronary artery from the left sinus, and 13 other anomalies. Patients with inter-arterial course between the great arteries presented more frequently with syncope and/or sudden death compared to patients without (23.4% vs. 0%, p = 0.026). Diagnosis was reached using first-line transthoracic echocardiography in 65% of cases. Surgical repair was performed in 61 patients (61%). All operated patients (60 direct implantations, 1 unroofing) had been diagnosed with inter-arterial course between the great arteries, and were asymptomatic at a mean (±SD) postoperative follow-up of 4.9 ± 5.3 years.

Conclusions: An inter-arterial course of the anomalous right or left coronary arteries arising from the opposite sinus is associated with life-threatening events. Direct reimplantation of coronary artery is reliable, and should be discussed even in asymptomatic patients.