Clinical Presentation and Therapy of Coronary Artery Anomalies

Abstract

A patient with anomalous origin of the left coronary artery from the pulmonary artery may present with signs and symptoms of myocardial infarction and congestive heart failure in infancy (or the condition may be detected serendipitously in adulthood or at autopsy). Anomalous origin of the left coronary artery from the right sinus of Valsalva is a rare but important malformation because it is associated with a significant risk of sudden death. It is unclear whether or not anomalous origin of the right coronary artery from the left sinus of Valsalva is associated with sudden death. Thus, the indications for operative repair of this lesion are less clear than when the left coronary artery arises anomalously from the right sinus of Valsalva.

Single coronary artery occurs in approximately 2 of every 1000 patients. It is associated with transposition of the great arteries, coronary artery fistula, and bicuspid aortic valve.

Coronary artery fistula constitutes 0.2–0.4 % of congenital cardiac defects. Fistulae originate equally from the right and left coronary arteries. Usually, the fistula connects to the right ventricular cavity. The right atrium is the second most common terminus, and two-thirds of fistulae draining into the right atrium originate from the right coronary artery.