Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: ? a 5(th) aortic arch with a systemic-to-pulmonary arterial connection

Major abnormalities of pulmonary circulation are uncommon in the patient wi th pulmonary atresia and intact ventricular septum. Non-confluent pulmonary arteries have only rarely been described in this setting. In this case rep ort, we describe a patient in whom the pulmonary arteries are non-confluent , with the right pulmonary artery supplied through a right-sided arterial d uct, and the left pulmonary artery most likely through a fifth aortic arch, thus providing a systemic-to-pulmonary arterial connection. We discuss the various forms of non-confluent pulmonary arteries in the setting of pulmon ary atresia and intact ventricular septum.