ABSTRACT
The congenital cardiac surgeon will learn early in his or her career that a problem related to a coronary artery is the commonest cause of an irretrievable situation developing in the operating room. Extracorporeal membrane oxygenation (ECMO) or a ventricular assist device may allow bridging to heart transplant but will not correct the problem. They may even exacerbate the problem.1 Thus it is critically important that the surgeon understands a child’s coronary anatomy and physiology preoperatively and the way in which that anatomy might be at risk during the planned procedure. For example, pulmonary atresia with intact ventricular septum is frequently complicated by coronary artery fistulas with or without coronary artery stenoses. Inappropriate decompression of the right ventricle can be a fatal event for these babies. Transposition of the great arteries can be complicated by unusual coronary ostial distribution and branching patterns. Patients with hypoplastic left heart syndrome who have the anatomic variant of aortic atresia with mitral stenosis also have a high incidence of coronary artery fistulas to the left ventricle. At least 5% of patients with tetralogy of Fallot have an anomalous anterior descending coronary artery arising from the right coronary artery. These various coronary problems are covered in the relevant chapters for the major anomaly. This chapter will focus on anomalies in which the coronary artery problem is the principal lesion. Interestingly, coronary anomalies are more common in females than males unlike the majority of congenital cardiac anomalies that are more common in males.2
