CAR-1. A life threatening rare congenital coronary artery anomaly associated with sudden cardiac death in young people.

Sudden cardiac death results in about 350,000 deaths annually in the US. Most common etiology in young people is hypertrophic cardiomyopathy followed by coronary artery anomalies and coronary artery disease. We describe a patient with a rare coronary artery anomaly that has been associated with sudden cardiac death. A 38-year-old white male, previously healthy, had symptoms of exertional chest pain of 6 months' duration. His younger sister had died of sudden cardiac death. He smoked two packs per day. Physical exam, laboratory data and chest radiograph was unremarkable. Electrocardiogram showed normal sinus rhythm. Due to a strong family history of sudden cardiac death, an exercise Cardiolite stress test was performed which revealed high-risk myocardial perfusion scan. Subsequently, diagnostic coronary arteriography revealed an anomalous origin of left main coronary artery arising from right sinus of Valsalva with an interarterial course between pulmonary artery and aorta. He also had high-grade coronary atherosclerotic lesions involving all vessels. He underwent successful coronary artery bypass grafting for prevention of sudden cardiac death and relief of anginal symptoms. The largest North American cohort reviewed more than 125,000 coronary arteriograms and found an incidence of 1.3% for coronary artery anomalies; 81% were benign, but others have been associated with potentially serious sequelae including myocardial infarction, syncope and sudden cardiac death. Ectopic coronary origin from the opposite aortic sinus or pulmonary artery, single coronary artery and large coronary fistulae are extremely rare but potentially serious anomalies that require accurate recognition and prophylactic surgical correction.

Michael Nillas, Silvestre Cansino, Mark Studeny, and Imran Arif. Department of Cardiology, Joan C. Edwards School of Medicine, Huntington, WV.