Abstract 4372945: The Japanese nationwide survey of the anomalous aortic origin of a coronary artery in children over 20 years

Background: Anomalous aortic origin of a coronary artery (AAOCA) is rare but is one of the most important causes of sudden cardiac death in children with a variety of presentations. Aim: To determine the clinical presentation, course, and outcome of AAOCA based on a Japanese nationwide survey. Methods: According to the Japanese nationwide survey of congenital coronary anomalies in 2021, AAOCA diagnosed at < 20 years old between 2001 and 2020 were reported from 38 institutions. We sent the questionnaire including the diagnosis, the diagnostic modalities, the clinical presentation, the clinical course, and the outcome, and collected the data. Results: We identified 107 patients with AAOCA, 70 RCA from the left coronary cusp (Rt-AAOCA), 30 LCA from the right coronary cusp (Lt-AAOCA), and 7 others, using coronary angiogram (45%), CT (22%), and echocardiography (20%). Of them, 42 (39%) patients presented with cardiac events (n=24); syncope, or cardiac arrest or chest pain (n=18), at 12.6 ± 3.8 years old and were diagnosed with Lt-AAOCA in 20, Rt-AAOCA in 17, and 5 in others. With a short interval, 31 (74%) patients underwent surgical intervention at 13.1 ± 4.0 years old. All symptomatic patients survived to 17.1 ± 6.2 years old without further cardiac events. Conversely, 65 asymptomatic patients were diagnosed much earlier age at 5.3 ± 4.1 years old either by screening (49%) or in the clinical management of Kawasaki disease (48%). Diagnoses were Rt-AAOCA in 53 (82%), Lt-AAOCA in 10 (15%), and others in 2. In these asymptomatic patients, 6 patients (2 Rt-AAOCA) and4 Lt-AAOCA ) underwent surgical intervention because of symptom presentation at follow-up in 3 and the unfavorable coronary anatomy in 3. All asymptomatic patients survived to 10.0 ± 5.3 years old without further cardiac events. Conclusions: Less than half of patients with AAOCA presented at school age with cardiac symptoms and underwent timely surgical intervention with good outcomes. On the other hand, more than half of patients with AAOCA were diagnosed without sympotom much earlier age with clinical management of Kawasaki disease as an important opportunity in Japan. Practical guidelines for the management of these asymptomatic patients must be established.