Coronary Artery–Right Atrium Fistula

A 1-month-old infant was referred to our hospital for clinical evaluation of a harsh continuous murmur at mid-sternal level. Chest radiography showed moderate cardiac enlargement. The electrocardiogram was normal. Trans-thoracic echocardiography showed moderate enlargement of the right cardiac chambers and the left coronary artery. Subxyphoid views demonstrated communication between the coronary tree and the right atrium, which suggested a fistula. Color-flow Doppler imaging showed a moderate shunt, and this led to the diagnosis of coronary artery–right atrium fistula. The echocardiographic diagnosis was confirmed by cardiac catheterization. The infant successfully underwent corrective cardiac surgery at 3 months of age.

Fig. 1 A and C) Angiography reveals the enlarged left coronary artery and the fistulous connection between the coronary artery and the right atrium (white arrow). B and D) Color-flow Doppler echocardiography demonstrates enlargement of the coronary artery (red asterisk) and shows the site of drainage of the fistulous mouth (red arrow).

Fig. 2 Operative photograph reveals the enlarged left coronary artery and the tortuous coronary artery–right atrium fistulous communications (arrowhead).