The Electrophysiology Experience in Patients With Ebstein's Anomaly Undergoing the da Silva Cone Repair at UPMC Children's Hospital of Pittsburgh.

INTRODUCTION: The da Silva Cone repair (CR) has demonstrated to be an effective intervention for Ebstein's anomaly (EA). Perioperative management can be complicated by arrhythmias, but previous studies showed improvement with preoperative catheter and intraoperative surgical ablation. The purpose of this study was to describe the evolving diagnostic protocol and perioperative findings at our center.

METHODS: All patients referred to UPMC Children's Hospital of Pittsburgh from February 2016 to December 2024 with EA who underwent CR were reviewed. An electrophysiology study (EPS) was performed for those with a history of Wolff-Parkinson-White, supraventricular tachycardia, ventricular tachycardia, unexplained palpitations, or syncope.

RESULTS: There were 135 patients (median age 7.8 years, range: 0.14-62 years), and 55 patients (41%) were < 5 years old at the time of CR. Seventy-two (53%) patients had a pre-operative arrhythmia, and 37 patients (27%) had a pre-operative EPS at our center before CR. Nineteen (51%) had a significant finding, and 12 (32%) received a transcatheter ablation. Intraoperative ablation was performed in 86 (64%) patients with cavotricuspid isthmus (CTI) ablation performed in 74 (55%), of which 24 (18%) had a CTI with additional targeted accessory pathway ablation. Postoperative wire studies were performed in 11 (8%). Since implementing our current electrophysiologic diagnostic algorithm, 7% had a follow-up arrythmia and only 1 underwent a postoperative EPS.

CONCLUSION: Pre-operative EPS, targeted surgical ablation, and follow-up bedside wire study in EA patients with accessory pathway-mediated arrhythmias can serve as an effective model in patients undergoing CR.