Cone Repair in a Non-Ebstein Disease Setting

This case was performed during a humanitarian mission in Paraguay in April 2023. The patient is a ten-year-old, 40 kg girl with severe tricuspid regurgitation. The surgical team found annular dilatation with no apical displacement, a large anterior leaflet, a restrictive posterior leaflet, and an absent septal leaflet with a remnant in the papillary muscle.

After a valve assessment, the surgical strategy was decided. The team proceeded with posterior leaflet delamination, anterior leaflet detachment, clockwise rotation of the anterior and posterior leaflets, cono-like bicuspid neo-valve arrangement, re-implantation of former posterior leaflet in the septal annulus, reinforcement of anteroseptal with a remnant of the septal leaflet, De Vega annular plication over a Hegar probe, and re-implantation of the former anterior leaflet. The ECC was 78 minutes and cross-clamp time was 55 minutes.

Lessons learned from the Cone technique are valuable tools in surgical scenarios different than Ebstein disease. In addition, classical maneuvers like De Vega annuloplasty help to fix the tricuspid valve in a comprehensive way.

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