Congenital coronary artery abnormalities predisposing to sudden cardiac death.

Isolated congenital coronary artery anomalies (CCAAs) are a rare but well-described cause of sudden cardiac death (SCD). Anomalous origin of a coronary artery from the opposite sinus has been most frequently associated with myocardial ischemia, ventricular arrhythmias, and sudden death, particularly when the anomalous coronary courses between the great arteries. Importantly, symptoms occur in only about 50% of people with CCAA who subsequently have SCD. Echocardiography is effective in prospectively identifying these coronary artery abnormalities but requires sophisticated echo equipment and a laboratory examination with targeted, high-quality imaging. Although surgical intervention for some forms of CCAA is feasible with good early results and is clearly indicated for symptomatic patients, no clear best practice has been established for the management of those identified serendipitously without symptoms.