Scimitar syndrome is a rare congenital anomaly essentially requiring surgical management. Nonsurgical interventional treatments are rarely possible and are associated with poor outcome. We report a case of Scimitar syndrome with obstructed partial pulmonary venous confluence, large patent ductus arteriosus, and severe pulmonary artery hypertension, where a significant decrease in the pulmonary artery pressure was achieved by pulmonary venous stenting in combination with coiling of aberrant vascular supply to the sequestered lung segment. However, the child succumbed to lower respiratory tract infection. To the best of our knowledge, no such procedure in combination has been reported in the literature. <Learning Objective: The percutaneous approach to deal with pulmonary hypertension in a case of Scimitar syndrome is discussed. The outcome of this case suggests that interventional procedures such as pulmonary venous stenting could be done as an emergent palliative treatment to relieve pulmonary hypertension.>.
Keywords: Pulmonary hypertension; Pulmonary venous obstruction; Pulmonary venous stenting; Scimitar syndrome.