Prosthetic valve replacement of the left-sided atrioventricular (AV) valve was performed
in a patient with AV discordance and Ebstein's anomaly of the left-sided AV valve
without other associated anomalies. An anatomical study of 22 specimens with AV discordance
did not show a case with similar anatomy; 5 hearts had mild degrees of downward displacement
of the attachment of the left-sided AV valve leaflets, with marked dysplastic changes
and variable degrees of hypoplasia. The surgical implications are different according
to the degree of downward displacement of the leaflets. This feature and the size
of the annulus and the morphologically right ventricle should be considered in preoperative
evaluation, along with the operative approach, possible presence of atrialization
of the wall of the proximal chamber and the relation of the left AV valve to the conduction
system. Preoperative diagnosis of the anomaly is possible by angiocardiography and
other methods.
Corrected transposition of great arteries - Atrioventricular discordance - Ebstein's
anomaly
