Background The anomalous origin of one pulmonary artery is a rare malformation, which so far has mainly been found as an anomalous origin from a different site of the aorta, accounting for 0.12% of all congenital heart diseases. This case report introduced a very rare case of the anomalous origin of one pulmonary artery which had never reported in the clinic. Case summary A 2-year-old boy with a 6-month history of shortness of breath and recurrent respiratory infection, was diagnosed left pulmonary artery (LPA) directly arising from the right ventricle by transthoracic echocardiography and multidetector computed tomography without a deletion in the region of 22q11. Eventually, the LPA was further conformed that arised from the right ventricle during the operation, and was corrected with a well clinical outcome. Discussion The surgical technique for repair of this anomalous LPA was not difficult in our case. However, the embryonic development of the present case still could not be completely explained by the current embryologic postulates since it was a new malformation that never reported. Due to its rarity, there is still much to learn about the origin and development of the pulmonary arteries that possibly develop prenatally.
