Congenital aneurysmal circumflex coronary artery fistula in a pregnant woman

Coronary artery fistula (CAF) is an abnormal communication between an epicardial coronary artery and a single or multiple cardiac chambers, large vessels or other vascular structures (1). Although it is a rare condition, it is the most frequent congenital coronary anomaly (2), and it accounts for 0.2-0.4% of congenital cardiac anomalies. Approximately 50% of pediatric coronary vasculature anomalies are coronary artery fistulae. Small fistulae remain clinically silent and are recognized during routine echocardiography or at autopsy. Larger fistulae enlarge progressively over time, and complications, such as congestive heart failure, arrhythmias, infectious endocarditis, aneurysm formation, rupture, and death, are more likely to arise in older patients. The most common feature is a right coronary artery fistula connecting to the right ventricle. Reports on the clinical repercussions of CAFs in pregnancy are uncommon (3,4). We describe a pregnant woman with a congenital aneurysmal circumflex coronary artery fistula to the right ventricle and discuss the appropriate management for this situation.