THE CONE RECONSTRUCTION OF EBSTEIN ANOMALY: A CASE REPORT

Abstract

Ebstein anomaly is a rare congenital defect in which origins of variably deformedposterior and septal leaflets of tricuspid valve are displaced downwards into rightventricle(failure of delamination, with characteristic sail-like anterior leaflet.Rotational displacement of tricuspid valve divides the right ventricle into proximal(atrialized) and distal (ventricularized) portions. Due to marked dilation of trueannulus and right ventricle, ebstein valves are usually regurgitant. Out ofnumerous reapir techniques, Da Silva's Cone reconstruction is different in that itis closest to anatomical repair in which there is mobilization of anterior andposterior tricuspid leaflets from their anomalous attachments in the rightventricle, with clockwise rotation of the free edge and sutured to the septal borderof anterior leaflet. The basic principle of this repair is to create a cone with its tipfixed at the right ventricular apex and the base sutured to the true tricuspid valveannulus level. We present a case of Ebstein anomaly in which Cone repair wasdone with uneventful recovery.