A 4 year old girl was admitted with cyanosis after exercise. Physical examination found a continuous murmur which was loudest in the upper right parasternal region. The ECG was normal. Transthoracic echocardiography revealed mild right ventricular and atrial dilatation; it also revealed a fistula originating from the left main coronary artery (LMCA), rounding the ascending aorta and left atrium, and emptying into the right atrium. Ascending aorta angiography showed the presence of an aneurysmal fistula originating from the left main coronary artery and draining into the right atrium. The diameter of the fistulous orifice was 3.0 mm (upper panel, middle column).
A 5 French JL3.0 angiography catheter was introduced into LMCA. Within the catheter a 0.014 inch guiding wire was inserted into left main coronary artery and crossed the fistula into the right atrium. The guiding wire was caught in the right atrium by the Amplatz “goose neck” snare, and passed through a femoral vein. Over the wire a Mullins sheath was transvenously advanced across the fistula (lower panel, middle column). Then the guiding wire was removed. An 8/6 mm Amplatzer duct occluder (ADO) was advanced within the sheath until it reached the tip. The delivery system was carefully withdrawn until the ADO was opened completely at the atrial end of the fistula (upper panel, right column). Post-deployment angiography showed there was no residual shunting, and the coronary blood flow improved (lower panel, right). The murmur disappeared.
A coronary artery fistula most commonly originates from the right coronary artery, but a fistula originating from the left main coronary artery is rare. Coronary artery fistulae can cause myocardial ischaemia, congestive heart failure, bacterial endocarditis, cardiac arrhythmia, and rupture of aneurysmal fistulae. Treatment options include surgical ligation and coil embolisation. An Amplatzer duct occluder provides another means of treatment.