title: Pulmonary stenosis development and reduction of pulmonary arterial hypertension in atrioventricular septal defect: a case report.
abstract: A 24-year-old patient was admitted for dyspnoea and syncope. He had a previous history of complete atrio-ventricular septal defect and trisomy 21. At the age of 6 months, in 1984, cardiac catheterization revealed a quasi-systemic pulmonary arterial hypertension with a bidirectional shunt corresponding to an Eisenmenger syndrome. Corrective cardiac surgery was not performed at this time because surgical risk was considered too high. Until the age of 20 years old, he showed few symptoms while under medical treatment. But since 2006, his functional status became worse with an increased dyspnoea, syncopes, and severe cyanosis. In these conditions, haemodynamic parameters have been re-evaluated in 2006 and 2008.They highlighted a late and progressive development of a valvular and infundibular pulmonary stenosis leading to a normalisation of pulmonary arterial pressures. At the age of 24 , the patient underwent corrective cardiac surgery which was successful. Late development of both infundibular and valvular pulmonary stenosis have not been described before in non operated congenital ventricular septal defects, but development of one or the other abnormality would be found in 8% of patients. The physiopathological mechanism of this obstruction is unclear. Nevertheless, in unoperated congenital cardiac shunt lesions, reversibility of severe pulmonary arterial hypertension should be reconsidered and re-assessed during follow up.
authors.affiliation: Clinique de cardiologie, CHU de Grenoble, BP 217, 38043 Grenoble cedex 09, France. emelinebarth@yahoo.fr
authors.forename: Emeline
authors.initials: E
authors.lastname: Barth
authors.forename: Hélène
authors.initials: H
authors.lastname: Bouvaist
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Marlière
authors.forename: Gérard
authors.initials: G
authors.lastname: Ninet
authors.forename: Gérald
authors.initials: G
authors.lastname: Vanzetto
mesh_terms: D004314:Down Syndrome / Q000150:complications; D004562:Electrocardiography; D006343:Heart Septal Defects / Q000150:complications* / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000503:physiopathology*; D017380:Hypertrophy, Right Ventricular / Q000000981:diagnostic imaging; D008297:Male; D011666:Pulmonary Valve Stenosis / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy; D016896:Treatment Outcome; D014463:Ultrasonography; D055815:Young Adult