Coronary Artery Anomalies and Coronary Artery Disease in Adults with Congenital Cardiac Disease

Asia Pacific Cardiology - Volume 1 Issue 1;2007:1(1):70-72


Congenital coronary artery abnormalities include various types of anomalies such as anomalous aortic origin, anomalous proximal course, anomalous distal connection, anomalous pulmonary arterial origin and anomalous coronary artery size. In this article I will review the most frequent abnormality and clinically significant anomalies: coronary artery fistulae and anomalous origin of the left coronary artery from the pulmonary trunk in adults. Recently, many patients with congenital heart disease (CHD) have reached adulthood and some of them are already in their mid-50s. I will touch on coronary artery sequelae attributed to antecedent Kawasaki disease observed in adult ischaemic heart disease, as well as the recent topic of coronary circulation in adults with cyanotic congenital heart disease (CCHD).

Coronary Artery Fistulae

Coronary artery fistulae are the most prevalent haemodynamically significant congenital anomalies of the coronary artery. Both coronary arteries arise from their assigned aortic sinus, but a fistulous branch of one or more of these arteries communicates directly with a cardiac chamber, pulmonary trunk, coronary sinus, vena cava or pulmonary vein. Adult survival is expected, but the lifespan may not be normal. The clinical manifestation depends largely on the amount of blood flowing through the fistula. Myocardial ischaemia may develop when the fistulous bypass induces coronary artery steel. In some cases of Kawasaki disease, this coronary artery anomaly is accidentally detected by a coronary angiogram.
Occasionally, a coronary artery fistula closes naturally.1 The long-term results of surgical repair are related to the volume of blood through the fistulous communication, the site of the communication and myocardial ischaemia before surgery. However, the results of late surgery are excellent in most cases. Surgery is usually safe; therefore, surgery or coil occlusion is usually recommended unless there is a single isolated fistula with a trivial shunt. Many coronary artery fistulae are very small (usually originating from the proximal left coronary artery and entering the pulmonary trunk).2


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