Ostial stenosis of anomalous origin of the left coronary artery from the pulmonary artery in an adult: Lives from constraints and dies from freedom

Abstract

An anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), a rare congenital anomaly, the enlarged right coronary artery supplies the left ventricle through retrograde collaterals before preferentially directing into the lower-pressure pulmonary artery system, resulting in coronary steal. The infant type is characterized by the absence of collateral vessels, and global myocardial ischemia is a major cause of death in infancy. If not treated, up to 90% of them will die during the 1st year of life. Myocardial ischemia or infarction left ventricular (LV) dysfunction with or without mitral regurgitation, life-threatening arrhythmias, or sudden cardiac death may all be manifestations of symptomatic adult-type ALCAPA. In those patients who have survived to adulthood without surgery, pathophysiological mechanisms enabling adequate LV perfusion, such as a large number of well-formed functioning collaterals, and in a few cases, the presence of ostial stenosis of the left coronary artery (LCA), provide selective survival advantage. We review the correlation between clinical presentation, pathophysiological findings, and angiographic features of ostial stenosis of LCA in the setting of adult-type ALCAPA.