title: [Pulmonary atresia with intact ventricular septum associated with severe aortic stenosis].
abstract: Pulmonary atresia with intact ventricular septum is the complete obstruction of the right ventricular outflow tract due to pulmonary valve atresia in the absence of ventricular septal defect. Pulmonary flow is dependent on the ductus arteriosus. Other morphological anomalies are also present. Aortic stenosis in association with pulmonary atresia with intact ventricular septum is extremely infrequent, and very few cases have been described. We report a 19-year-old primiparous woman in whom the fetal echocardiogram showed pulmonary atresia with intact ventricular septum. The neonate had low oxygen saturation and a systolic murmur originating in the aorta. An echocardiogram showed pulmonary atresia with intact ventricular septum and a bicuspid, thickened, stenotic aortic valve. Low cardiac output ensued and could not be controlled, and the infant died.
authors.affiliation: Servicio de Cardiología. Hospital Universitario Puerta de Hierro. Madrid. Spain. robertoperaira@hotmail.com
authors.forename: Juan R
authors.initials: JR
authors.lastname: Peraira
authors.forename: Margarita
authors.initials: M
authors.lastname: Burgueros
authors.forename: Isabel
authors.initials: I
authors.lastname: Esteban
authors.forename: Luis
authors.initials: L
authors.lastname: García-Guereta
authors.forename: María D
authors.initials: MD
authors.lastname: Rubio
authors.forename: Francisco J
authors.initials: FJ
authors.lastname: Alvarez-Ferreira
mesh_terms: D000328:Adult; D001024:Aortic Valve Stenosis / Q000150:complications / Q000175:diagnosis*; D005260:Female; D006801:Humans; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000175:diagnosis*; D018633:Pulmonary Atresia / Q000150:complications / Q000175:diagnosis*; D012720:Severity of Illness Index