title: Total pulmonary artery atresia associated with abnormal pulmonary venous drainage as a rare presentation of scimitar syndrome.
abstract: Scimitar syndrome or pulmonary venolobar syndrome is a rare, complex, and variable malformation of the right lung characterized by an abnormal right sided pulmonary drainage into the inferior vena cava, malformation of the right lung, abnormal arterial supply, and sometimes cardiac malformation. Despite the varying degrees of pulmonary hypoplasia and pulmonary artery hypertension, about half of the patients with scimitar syndrome are asymptomatic or mildly symptomatic when the diagnosis is made. Neonates have severe symptoms and worse prognosis while older children come to light because of recurrent respiratory infections, heart murmur, or an abnormal chest radiograph.
authors.affiliation: Department of Radiology, Faculty of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran.
authors.forename: Reza
authors.initials: R
authors.lastname: Javadrashid
authors.forename: Mahmood
authors.initials: M
authors.lastname: Samadi
authors.forename: Homa
authors.initials: H
authors.lastname: Akbari
keywords: CT Angiography; Pulmonary Artery Atresia; Scimitar Syndrome