Right Ventricular Outflow Tract Obstruction in Ebstein's Anomaly: a Caveat in Echocardiographic Diagnosis

Abstract

It is critical to distinguish between anatomic [obstructive anterior tricuspid valve leaflet (ATL) or pulmonary atresia (PA)] and functional (tricuspid insufficiency with elevated pulmonary vascular resistance) right ventricular outflow tract (RVOT) obstruction in cyanotic infants with Ebstein's anomaly (EBS). We reviewed 4 cases of EBS to determine the accuracy of 2-D echo in distinguishing anatomic from functional RVOT obstruction. Two of 4 were newborns. The other 2 were children (8; 12 y.o.) with severe cyanosis as neonates, requiring Waterston shunt. EBS with PA was the 2-D echo diagnosis in the 8 y.o., and an obstructive ATL in the 12 y.o. Both were confirmed at operation. The 2 newborns had 2-D echo findings suggesting obstructive ATL with minimal pulmonary valve (PV) motion. Both had shunt operation. One died, and, at autopsy, the ATL was found to be non-obstructive. The other had a 2-D echo 1 month post-operatively, revealing only mild pulmonary valve thickening.In cyanotic neonates with severe forms of EBS, functional RVOT obstruction can be mistaken for anatomic by 2-D echo. Apparently obstructive ATL and minimal PV motion could be the result of reduced antegrade blood flow. Prior to operation, presumed anatomic obstruction should be confirmed by cardiac catheterization and angiocardiography.

DOI: 10.1203/00006450-198404001-00144

Cite this paper

@article{Alboliras1984RightVO, title={Right Ventricular Outflow Tract Obstruction in Ebstein's Anomaly: a Caveat in Echocardiographic Diagnosis}, author={Ernerio T. Alboliras and David J . Driscoll and Donald J. Hagler}, journal={Pediatric Research}, year={1984}, volume={18}, pages={117A-117A} }