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
Landau, J.F.; Durand, M.; Vincent, P.; Guilmet, D.; Delamare, J.
Archives des Maladies du Coeur et des Vaisseaux 76(2): 233-237
1983
ISSN/ISBN: 0003-9683 PMID: 6407431 Document Number: 217950
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.