AORPA: A Rare Congenital Heart Disease

The anomalous origin of the pulmonary artery from the ascending aorta (AOPA) is also referred as "hemitruncus" and accounts for 0.1% of all congenital heart diseases. Anomalous origin of pulmonary artery (AOPA) from the ascending aorta is an unusual and critical cardiovascular anomaly, which frequently involves the right pulmonary artery (RPA). Hemitruncus occurs alone in roughly 60% of cases of right pulmonary artery abnormality, which is 6 times more frequent than left. The most common related condition is patent ductus arteriosus, however tetralogy of Fallot, an atrial septal defect, a ventricular septal defect, and coarctation of the aorta have also been described. The known underlying aetiology of this anomaly is a partial or total developmental failure of the left sixth arch, despite the fact that the pathophysiology of this anomaly is poorly understood. As a result, there is a significant left-to-right shunt, which causes pressure and/or volume overload in both lungs. One lung receives the whole cardiac output from the right ventricle, while the other receives blood from the aorta at a systemic pressure.