Anomalous aortic coronary artery origin: Surgical, anesthetic and echocardiographic considerations. A case report and comprehensive review

Introduction

Anomalous aortic origin of a coronary artery (AAOCA) is an uncommon congenital cardiac anomaly with an estimated prevalence of 0.01 % to 2 % that may occur in isolation or with other congenital cardiac anomalies. Anomalous aortic origin of the right coronary artery (AAORCA) from the opposite sinus of Valsalva is 4 to 5 times more common than anomalous aortic origin of the left coronary artery (AAOLCA) from the opposite sinus of Valsalva. However, SCD and symptoms of ischemia have been more commonly associated with the latter. In a large autopsy study, an anomalous aortic origin of a coronary artery (AAOCA) was the second most common cause of SCD in young competitive athletes with the left CA being most frequently implicated. The majority of deaths occurs during or shortly after peak exercise and may be the first presenting symptoms.

Case description

We present the case of a 15-year-old male presenting with exertional angina secondary to an anomalous right coronary artery (RCA) originating from the left sinus of Valsalva with an intramural and interarterial course. We describe the diagnostic imaging, surgical management, and anesthetic considerations for the successful surgical repair of this complex anomaly using intramural unroofing and main pulmonary artery (MPA) translocation. The role of intraoperative two and three-dimensional (2-D, 3-D) transesophageal echocardiography (TEE) is highlighted.

Summary discussion

Management guidelines for CAAs have been published by the American and European cardiology societies. Diagnostic and therapeutic decision-making algorithms incorporate the delineation of abnormal anatomy as well as functional assessment of ischemic burden and arrhythmia testing. Therapeutic decisions should be personalized to patient characteristics based on specific anatomy and the demonstration of a causal link between the CAAs and myocardial ischemia. Perioperative management of CAA presents unique challenges due to the dynamic mechanism of ischemia. Several surgical options exist depending on the anatomic variant. Standardization of care is especially important in rare diseases. Surgical management of AAOCA has evolved over time, and can be achieved with low morbidity and mortality. The establishment of coronary artery programs in highly specialized centers has streamlined care.