Right pulmonary artery to left atrial fistula is classified based on the right pulmonary artery branching, individual right pulmonary venous drainage, and presence of an aneurysmal segment. A rare association with scimitar syndrome and right lung devoid of blood supply from right pulmonary artery is described in this report. The anatomical and management differences between the different types are highlighted.
Keywords: Right pulmonary artery to left atrium fistula; abnormal pulmonary venous drainage; device closure; lung hypoplasia.