[Hypoplasia of the right ventricle and cyanosis due to left-right atrial shunt within the framework of Turner's syndrome. Surgical closing of the interatrial connection after occlusion testing with balloon catheterization].
The authors describe the unusual cardiac malformation in a young girl with Turner's syndrome: right ventricular hypoplasia and atrial septal defect, resulting in central cyanosis. A trial of temporary occlusion of the atrial septal defect at cardiac catheterisation before surgical closure suppressed the right-to-left shunt and the cyanosis without significantly changing the hemodynamic parameters. The authors review the types of cardiovascular abnormalities associated with Turner's syndrome and emphasize the value of a temporary occlusion test of defects in certain cyanotic cardiac malformations with atrial right-to-left shunts; it may lead to a relatively simple surgical procedure, as in the case described.
D000328:Adult; D006328:Cardiac Catheterization; D003490:Cyanosis / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000150:complications; D006352:Heart Ventricles / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D011859:Radiography; D014424:Turner Syndrome / Q000150:complications* / Q000175:diagnosis / Q000601:surgery
Hypoplasie du ventricule droit et cyanose par shunt droite-gauche auriculaire dans le cadre d'un syndrome de Turner. Fermeture chirurgicale de la communication interauriculaire après test d'occlusion par ballonnet au cours du cathétérisme.