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A premature newborn male (29th week gestation, birth weight 890 g) was referred for investigation of a heart murmur. Echocardiography showed a double outlet right ventricle with hypoplastic left ventricle, ventricular septal defect, critical subvalvar and valvar stenosis without detectable flow across the aortic valve, left sided interrupted aortic arch type B, ductus, persistent left superior vena cava draining into the coronary sinus. In addition, an abnormal vessel between the main pulmonary artery and the aortic root was seen, which showed a flow from the pulmonary artery to the ascending aorta. This led to the differential diagnosis of an aortopulmonary window or a coronary fistula.
To clarify the diagnosis, cardiac catheterisation was performed from the femoral vein (4 F sheath). Pulmonary angiography showed a coronary fistula with contrast flow from the main pulmonary artery (PA) to the aorta. A selective injection into the pulmonary orifice of the fistula in a laid back view clearly showed the contrast flow through the fistula of the left coronary artery into the ascending aorta (AAo) and subsequently the right brachiocephalic trunk and left common carotid artery (fig). An angiographic run after blocked injection into the main pulmonary artery revealed a relatively small left atrium, a restrictive foramen ovale, and a double outlet right ventricle with functional mitral and aortic atresia.
In this patient a coronary artery fistula between the aorta and pulmonary artery was the only source of antegrade perfusion into the ascending aorta in functional aortic atresia and interrupted aortic arch through retrograde coronary artery flow.