Abstract

Right pulmonary agenesis (PA) with left pulmonary artery sling (LPAS) is a very rare vascular anomaly commonly associated with tracheobronchial anomalies. It is also frequently linked with cardiac and extra cardiac defects. Associated major cardiac and tracheobronchial anomalies determine patients’ surgical prognosis. Fetuses with PA and LPAS prenatal diagnosis require echocardiograhic screening to rule out intra and extra cardiac lesions which worsen prognosis. We report a case of prenatal diagnosis of right pulmonary agenesis with LPAS and total pulmonary venous return. To our knowledge, this malformation has never been reported as an associated cardiac defect.