The anesthetic management and physiologic implications in infants with anomalous left coronary artery arising from the pulmonary artery

摘要

ANOMALOUS ORIGIN of the left coronary artery from the pulmonary artery (ALCAPA) is an uncommon congenital heart defect with an incidence of approximately 1 in 300,000 live births. The presentation and onset of symptoms typically occur shortly after the neonatal period as the pulmonary vascular resistance falls and left coronary blood flow diminishes. Decreased coronary blood flow results in myocar-dial ischemia, tissue infarction, mitral insufficiency, and dilated ischemic cardiomyopathy. Approximately 10% to 20% of patients with this condition remain asymptomatic and survive to adulthood.1'2 They can then present with coronary ischemic syndromes or sudden death. The diagnosis is made with echo-cardiography and, occasionally, cardiac angiography. Surgery is indicated in nearly all children and should be done before the development of severe myocardial dysfunction. Anesthetic management must be meticulous in the perioperative period because infants with an anomalous coronary artery are at a significantly increased risk of myocardial ischemia, cardiac arrest, and sudden cardiac death.