Da Silva’s Cone Repair and Repair of an Anomalous Left Upper Pulmonary Vein Return

A 37-year-old female presented with a history of Ebstein anomaly of the tricuspid valve with severe tricuspid regurgitation (TR), a severely dilated right atrium, an atrial septal defect, partial anomalous pulmonary venous return, and WPW ablation on three occasions. Cardiac MRI and echocardiogram showed apical displacement of the hinge point of the septal leaflet of the tricuspid valve consistent with Ebstein's anomaly with severe TR. The partial anomalous pulmonary venous return involved the left upper pulmonary vein draining into the innominate vein. Her case was discussed, and the consensus was for surgical indication. 

During surgery, the findings included a severely dilated and distended right atrium. The tricuspid valve’s Ebstein’s annulus was severely rotated. The anterior leaflet and the inferior leaflet were severely tethered to the anterolateral wall of the right ventricle. The septal leaflet was hypoplastic and severely displaced downward consistent with type C Ebstein's anomaly. The anomalous upper pulmonary vein was passing behind the right pulmonary artery and draining into the innominate vein. There was an 8 mm foramen oval type of atrial septal defect. The authors performed Da Silva’s cone repair of the tricuspid valve with septal leaflet augmentation with autologous pericardium. This repaired the anomalous left upper pulmonary vein return (the anomalous left upper pulmonary vein was divided near its distal connection to the innominate vein, beveled, and anastomosed to the left atrial appendage) and closed the atrial septal defect. The authors then performed a maze procedure. The patient’s postoperative management was satisfactory, and she was discharged four days after surgery. 

References

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5. Da Silva JP, Viegas M, Castro-Medina M, Da Fonseca Da Silva L. Da Silva Cone operation after the Starnes procedure for Ebstein’s anomaly: new surgi- cal strategy and initial results. J Thoracic Cardiovasc Surg Tech. 2020;3: 281-3.
6. Luciana Da Fonseca Da Silva, MD, Jose Pedro Da Silva, MD, Laura Seese, MD, MS, Albert F. Guerrero Becerra, MD, Mario Castro-Medina, MD, Melita Viegas, MD, and Victor O. Morell, MD, Pittsburgh, Pa Cone repair after tricuspid valve replacement in Ebstein anomaly. JTCVS Techniques 2023;-:1-3 2666-2507

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