title: Scimitar syndrome with absent right pulmonary artery and severe pulmonary hypertension treated with coil occlusion of aortopulmonary collaterals in a term neonate.
abstract: Scimitar syndrome (SS) is a rare congenital malformation with an estimated incidence of approximately 2 in 100 000 births. A wide clinical spectrum is observed in children with this syndrome. The common clinical presentation in infancy is respiratory distress and tachypnoea due to associated pulmonary hypoplasia, pulmonary overcirculation and/or pulmonary hypertension. Babies with SS presenting with cardiac failure are prone to develop exaggerated pulmonary vascular disease. Hence early intervention, using either coil embolisation or surgical intervention, is indicated. We are reporting a case of a term baby boy who presented with respiratory failure during the first 24 h of life. Echocardiogram and CT angiogram revealed SS. The baby needed intubation due to respiratory failure. Aortopulmonary collaterals, identified on aortic angiogram, were successfully occluded with detachable coils.
authors.affiliation: Department of Neonatology, Hamad Medical Corporation, Doha, Qatar Department of Pediatrics, Weill Cornell Medical College, Doha, Qatar.
authors.forename: Hussain
authors.initials: H
authors.lastname: Parappil
authors.affiliation: Department of Pediatric Cardiology, Hamad Medical Corporation, Doha, Qatar.
authors.forename: Faraz
authors.initials: F
authors.lastname: Masud
authors.affiliation: Department of Neonatology, Hamad Medical Corporation, Doha, Qatar Department of Pediatrics, Weill Cornell Medical College, Doha, Qatar.
authors.forename: Husam
authors.initials: H
authors.lastname: Salama
authors.affiliation: Department of Neonatology, Hamad Medical Corporation, Doha, Qatar Department of Pediatrics, Weill Cornell Medical College, Doha, Qatar.
authors.forename: Sajjad
authors.initials: S
authors.lastname: ur Rahman
mesh_terms: D006328:Cardiac Catheterization / Q000379:methods; D003097:Collateral Circulation; D017023:Coronary Angiography; D004232:Diuretics / Q000008:administration & dosage; D004452:Echocardiography; D005665:Furosemide / Q000008:administration & dosage; D006333:Heart Failure / Q000000981:diagnostic imaging* / Q000209:etiology / Q000628:therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000628:therapy; D007231:Infant, Newborn; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities / Q000000981:diagnostic imaging* / Q000503:physiopathology; D012127:Respiratory Distress Syndrome, Newborn / Q000209:etiology*; D012587:Scimitar Syndrome / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D004232:Diuretics; D005665:Furosemide