Anomalous left coronary artery from the pulmonary artery (ALCAPA), or Bland-White-Garland syndrome, is a rare congenital coronary anomaly that results in altered myocardial perfusion and a left to right shunt. It occurs in 1:300000 live births and represents 0.24% to 0.46% of all congenital cardiac diseases. Despite its rarity, it is one of the most common causes of ischemia and infarction in children. Ninety percent of these patients will die within the first year of life if untreated and diagnosing this abnormality in adulthood is extremely rare. Of those patients who survive to adulthood, the average age of sudden cardiac death is 35 years. The initial symptoms of the adult presentation vary widely from progressive dyspnea to sudden cardiac death; therefore, immediate surgical correction is highly recommended upon diagnosis. Understanding the pathophysiology and nature of collateral coronary flow in this congenital anomaly is paramount to the safe anesthetic management of adults with ALCAPA. Here we describe the intraoperative management and echocardiographic findings in a 68-year-old with with recently diagnosed ALCAPA undergoing surgical repair.
Keywords: cardiac anesthesia; cardiac surgery; children; congenital heart disease; intraoperative transesophageal echocardiography; ischemic mitral regurgitation; pulmonary artery pressure; transesophageal echocardiography.