Unusual catheter course in aortic arch atresia associated with aortopulmonary window

During catheterization of the aortic arch, the passage of an arterial catheter from the femoral artery to the aortic root is assumed almost axiomatically to indicate the integrity of the aortic arch. We will describe in this report a patient who demonstrated this catheterization finding but who did not have an intact aortic arch, thereby disproving this diagnostic criterion. The patient was a nine-day-old female, the product of a normal pregnancy and delivery. She was well until eight days of age, when cardiac failure became apparent and she was transferred to this hospital. Pulse rate was 148/minute and blood pressure was 50 mm Hg (right arm, flush). The brachial pulses were slightly decreased in amplitude and the femoral pulses were barely palpable. There was moderate cyanosis and an increased but not hyperdynamic right ventricular impulse. The first sound was normal. The second sound was of normal intensity and narrowly split. A grade 2/6 systolic ejection murmur was audible at the lower left sternal border. The liver was palpable at 7 cm below the right costal margin. Chest X-ray showed the heart moderately enlarged and there was increased pulmonary vascularity. The electrocardiogram showed moderate right ventricular hypertrophy. The course of the venous catheter from the right femoral vein to the right atrium was normal. It could be passed across a patent foramen ovale to the left atrium and left ventricle. It could also be manipulated to the right ventricle and then via the pulmonary artery through a ductus to the descending aorta.