title: Double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect.
abstract: Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle. This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. We report the case of an 18-day-old infant with DORV and extremely rare anatomical features, such as anterior and left-sided aorta and subpulmonary ventricular septal defect (VSD). In addition to the anatomic features, the role of the echocardiogram for guiding the diagnosis and the surgical therapy of this congenital heart disease are discussed.
authors.affiliation: Centro de Cardiologia Não Invasiva de São Paulo, São Paulo, SP, Brazil.
authors.forename: L B
authors.initials: LB
authors.lastname: Peixoto
authors.forename: S M
authors.initials: SM
authors.lastname: Leal
authors.forename: C E
authors.initials: CE
authors.lastname: Silva
authors.forename: S M
authors.initials: SM
authors.lastname: Moreira
authors.forename: J
authors.initials: J
authors.lastname: Ortiz
mesh_terms: D001011:Aorta / Q000002:abnormalities; D004310:Double Outlet Right Ventricle / Q000000981:diagnostic imaging*; D004452:Echocardiography; D006345:Heart Septal Defects, Ventricular / Q000000981:diagnostic imaging*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities; D014188:Transposition of Great Vessels / Q000000981:diagnostic imaging