Two children with an anomalous origin of one pulmonary artery from the inno
minate artery are reported. One was a 15-month-old boy presenting with resp
iratory distress. He had a right aortic arch and his left pulmonary artery
originated from the innominate artery. The other was a 1-month-old girl pre
senting with congestive heart failure. She had a left aortic arch and her r
ight pulmonary artery originated from the innominate artery. An understandi
ng of the embryological pathogenesis of these anomalies has significant the
rapeutic implications.