Two Surgical Strategies for Correction of Anomalous Origin of the Right Coronary Artery in Young Adults

Objectives: In this video, the authors demonstrate the surgical techniques for correction of anomalous aortic origin of the right coronary artery (RCA) with an interarterial and intramural course (AAORCA) or from the pulmonary artery (ARCAPA) in two young adults.

Case 1: A 16-year-old boy presented with a long-standing history of chest discomfort, palpitations, and dizziness with exercise. He felt anxious when these events occurred and had limited his activity levels. An electrocardiogram and echocardiogram were unremarkable. On exercise testing, he remained asymptomatic and tolerated 20 minutes of exercise using the standard Bruce protocol. However, he was noted to have 1 mm ST depression in the inferior leads during recovery, which was resolved after 30 minutes of rest. High resolution CT scan revealed an AAORCA, with a high take off of the RCA from the sino-tubular junction of the ascending aorta, and an inter-arterial and intra-mural course. After opening the pericardium, the abnormal course of the RCA was searched and visualized as a bulge on the aortic wall. The ascending aorta was opened longitudinally. The RCA origin arose anomalously from above the commissure between the left and right aortic sinus, and had an aberrant interarterial and intramural course. The patient underwent surgical de-roofing of the intramural RCA. He made an uneventful recovery and continues to be well and symptom-free at 3 years of follow up.

Case 2: A 25-year-old man presented with mild symptoms of chest discomfort with heavy exercise. Echocardiography demonstrated a RCA coming off the main pulmonary artery (MPA) and moderately reduced left ventricular function. CT angiogram confirmed the diagnosis showing the interarterial course of RCA between the aorta and main pulmonary artery. Cardioplegic arrest was performed with antegrade blood cardioplegia in both the aortic root and MPA. The MPA was incised, and the location of the orifice of the ARCAPA was identified. A coronary button was created and directly transferred into the anterior aspect of the ascending aorta. The MPA was then reconstructed using autologous pericardium. The patient had an uneventful recovery and is asymptomatic at 2 years of follow up.

Conclusion: Coronary arterial anomalies are the second most common cause of sudden cardiac death in competitive athletes, and account for 19% of sudden death in young athletes. The high-incidence of cardiac events in athletes may relate to arterial compression of the anomalous coronary artery and interference in diastolic coronary perfusion in increased cardiac output states. The surgical management of patients with anomalous origin of RCA includes unroofing of the anomalous artery, direct translocation, ostioplasty or bypass grafting, with good medium-term outcomes. It is imperative to have a high index of suspicion of exertional symptoms in young adults and to investigate thoroughly, so that a catastrophic event may be avoided.