American Association of Plastic Surgeons

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The Cone Operation for Ebstein's Anomaly is not Suitable for every infant
Christopher K. Knott-Craig, MD FACS, Umar Boston, MD, TK Susheel Kumar, MD.
Le Bonheur Children's Hospital, Memphis, TN, USA.

Objective: The Cone operation is currently the preferred method of repairing Ebstein's Anomaly (EA) in children and adults. This operation has recently been extended to infants, although the morphology may be significantly different from their older counterparts.
Methods: A three month old infant with severe EA and a PDA underwent a cone operation and bidirectional Glenn anastomosis. There was complete linear attachment of the lateral 2/3 of the anterior leaflet and the entire posterior leaflet. The posterior leaflet was completely delaminated and fenestrated as part of the Cone repair. A good repair was achieved with mild residual tricuspid regurgitation.
Results: Six hours after surgery the patient coded and was placed on ECMO through the chest. The new right ventricle had developed a massive aneurismal sac which was compressing the left ventricle and causing severe mitral regurgition. The patient was returned to the operating room and the entire right ventricle other than the infundibulum was resected; the atrial septum was re-opened and a fenestrated patch was sewn over the cone repair. The postoperative course was hazardous but ultimately the patient was discharged and remains well a year later. A video of the surgery will be presented.
Conclusions: Neonates and young infants with EA may not be candidates for the Cone Operation. Taking down the posterior tricuspid leaflet should be avoided in most cases.


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