title: Congenital right pulmonary artery agenesis with atrial septal defect and pulmonary hypertension.
abstract: Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7-year-old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.
authors.affiliation: Department of Pediatric Cardiology, Dr. Sami Ulus Pediatric Research and Training Hospital, Ankara, Turkey.
authors.forename: Utku Arman
authors.initials: UA
authors.lastname: Orun
authors.forename: Osman
authors.initials: O
authors.lastname: Yilmaz
authors.forename: Meki
authors.initials: M
authors.lastname: Bilici
authors.forename: Selmin
authors.initials: S
authors.lastname: Karademir
authors.forename: Cigdem
authors.initials: C
authors.lastname: Uner
authors.forename: Filiz
authors.initials: F
authors.lastname: Senocak
authors.forename: Vehbi
authors.initials: V
authors.lastname: Dogan
mesh_terms: D000015:Abnormalities, Multiple; D000959:Antihypertensive Agents / Q000627:therapeutic use; D000077300:Bosentan; D006328:Cardiac Catheterization; D002648:Child; D004417:Dyspnea / Q000209:etiology; D004452:Echocardiography; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging; D013449:Sulfonamides / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan