THE CONE RECONSTRUCTION OF EBSTEIN ANOMALY: A CASE REPORT

Ebstein anomaly is a rare congenital defect in which origins of variably deformed posterior and septal leaflets of tricuspid valve are displaced downwards into right ventricle(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 true annulus and right ventricle, ebstein valves are usually regurgitant. Out of numerous reapir techniques, Da Silva's Cone reconstruction is different in that it is closest to anatomical repair in which there is mobilization of anterior and posterior tricuspid leaflets from their anomalous attachments in the right ventricle, with clockwise rotation of the free edge and sutured to the septal border of anterior leaflet. The basic principle of this repair is to create a cone with its tip fixed at the right ventricular apex and the base sutured to the true tricuspid valve annulus level. We present a case of Ebstein anomaly in which Cone repair was done with uneventful recovery.