Cone repair and right ventricular resection in an adult patient with Ebstein's anomaly

Ebstein's anomaly is a rare congenital heart defect characterized by tricuspid valve malformation and right ventricular myopathy. The "cone procedure," introduced by Dr. José Pedro da Silva, represents a major advancement in the repair of Ebstein's anomaly, achieving near-anatomical tricuspid valve reconstruction. We describe the case of a 23-year-old-female with Carpentier type B Ebstein's anomaly, severe tricuspid regurgitation and Wolff-Parkinson-White syndrome, who underwent cone reconstruction combined with right ventricular resection. Detailed preoperative imaging guided the procedure, ensuring precise leaflet mobilization, delamination and cone creation. The atrialized right ventricular portion was reduced via triangular resection, preserving right ventricular geometry. An annuloplasty ring stabilized the repair. Postoperatively, the patient demonstrated excellent recovery, with trivial regurgitation and preserved right ventricular function confirmed at the one-year follow-up. This case highlights the reproducibility and effectiveness of the cone repair, supporting its role as the primary surgical approach for Ebstein's anomaly. Key factors for success include meticulous leaflet delamination, ensuring a complete 360° cone structure and maintaining RV geometry. Although long-term outcomes require further evaluation, the cone procedure offers superior valve competence and symptom relief compared to traditional repairs, minimizing the need for reoperation.