Repair of Anomalous Coronary Artery From the Pulmonary Artery by Aortic Implantation

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), a rare congenital cardiac lesion, is an important cause of myocardial ischemia and infarction in children, and carries a high mortality in the first year of life. In the current era, repair of ALCAPA is most commonly performed by aortic implantation. Coronary elongation techniques are available for cases where the ectopic coronary artery originates at point in the pulmonary artery that is distant from the aortic root. In cases where clinically significant mitral regurgitation (MR) is present mitral valve repair is usually not performed. The early outcomes for aortic implantation of ALCAPA are excellent in the current era, with survival rates exceeding 90%. A small number of children, those who present with cardiogenic shock and very poor ventricular function, may require post-repair mechanical circulatory support using ECMO or LVAD and still have high survival rates and expect good long-term recovery. Following repair of ALCAPA by aortic implantation, ventricular functional parameters such as depressed ejection fraction, ventricular dilation and MR should recover within 8 months of repair.