Abnormal origin of right pulmonary artery from the ascending aorta in an infant (" Hemitruncus")

Abstract

Key clinical message: Anomalous origin of right pulmonary artery from the ascending aorta is a rare congenital malformation and it needs surgical management. Consequences of this condition affect lead to pulmonary hypertension and severe pulmonary vascular disease.

Abstract: Anomalous origin of right pulmonary artery from the ascending aorta is a rare congenital heart malformation that results in early infant mortality affecting the right pulmonary artery more than the left. These patients are at risk for the early development of significant pulmonary hypertension. The surgical management during the early period of life is imperative.

Keywords: anomalous pulmonary artery; ascending aorta; congenital heart disease; hemitruncus arteriosus; pulmonary hypertension.

FIGURE 1

(A): Short axis echocardiographic view depicting the main pulmonary artery (MPA). Only the left pulmonary artery (LPA) originates from MPA. (B): Long axis view showed a vessel that arose lateral and posterior from the ascending aorta. (C): Short‐axis view showed a flow in the location of the right pulmonary artery but no vessel connected to the trunk of the pulmonary artery. (D): CT angiography showed the connection ascending aorta and right pulmonary artery. (E): CT reconstruction showed the complete anatomy of malformation. (F): Intraoperative image. (G): Postoperative echocardiography showing the good flow pattern through the RPA. (H): Postoperative CT showing the good surgical result. RPA is connected to MPA.