Clinical characteristics and outcomes of the anomalous aortic origin of a coronary artery: a nationwide pediatric survey in Japan.

An anomalous aortic origin of a coronary artery (AAO-CA) is one of the leading causes of sudden cardiac death in children. This study aimed to characterize the clinical features and outcomes of AAO-CA diagnosed between 2000 and 2019 using data from a nationwide survey in Japan. We identified 106 pediatric patients with AAO-CA (71 AAO-right CA and 35 AAO-left CA cases) from 32 participating institutions, with a median age of 9.1 (4.9-13.3) years at the time of diagnosis. At diagnosis, more than half (59%) of the patients were asymptomatic; AAO-CA was mainly detected during cardiac screening (52%) or Kawasaki disease management (48%), and the remaining patients (41%) presented with cardiac events (n = 24), chest pain (n = 15), or other manifestations (n = 4). Multivariable logistic regression analysis identified older age at diagnosis, AAO-left CA, and intra-mural coronary course as predictors of symptoms. Soon after established diagnosis [median of 2.0 (0.9-5.2) months], 7 asymptomatic and 32 symptomatic patients underwent surgical intervention, based on symptoms, anatomy, or positive myocardial stress test results. All patients (including one who presented with cardiopulmonary arrest and later underwent heart transplantation), survived to the age of 12.2 (7.4-16.2) years without further cardiac events. In conclusion, more than half of the patients with AAO-CA were diagnosed without symptoms with specific clinical features in Japan. Patients who underwent timely surgical intervention achieved excellent outcomes, highlighting the need for practical guidelines for the management of asymptomatic patients.