Congenital arterio-venous or arterio-arterial fistulae of the coronary arteries are direct connections between a branch of coronary artery and the lumen of one of the four cardiac chambers, coronary sinus, the superior vena cava, the pulmonary artery or the pulmonary veins without intervening capillary system. The incidence is accepted as 1:50000 live births. In 42% of cases the fistula is originated from the left coronary artery and left atrium drainage is seen only in %5 of the cases.
Giant coronary arteriovenous fistulae originating from circumflex coronary artery that was 24 x 40 mm in diameter and draining into the left atrium was diagnosed in a 57 year old man admitted to our emergency department with severe palpation and congestive heart failure. The fistulae was ligated with cardiopulmonary bypass. The occluded fistulae andnormal coronary anatomy were seen in the postoperative coronary angiography.