Right pulmonary artery originating from the ascending aorta with tetralogy of fallot and pulmonary atresia.

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

ABSTRACT: Anomalous pulmonary artery originating from the ascending aorta (often called hemitruncus) is a rare congenital cardiac defect requiring immediate management in the neonatal period. We report a case of a rare variant of anomalous right pulmonary artery originating from the ascending aorta in combination with pulmonary atresia and tetralogy of Fallot. The above-mentioned combination makes the surgical management of such cardiac defect exceedingly demanding.