Abstract

Patients with Ebstein’s anomaly (EA) who undergo tricuspid valve (TV) surgery may receive cardiac implantable electronic devices (CIED) for arrhythmias. Optimal CIED management and outcomes are unknown. To characterize CIED management and outcomes in patients with EA. Patients seen at the Mayo Clinic from 1972-2020 with EA and CIED were reviewed. Clinical, TV surgery and CIED details, complications, and lead parameters were recorded. Serial echocardiograms were reviewed for tricuspid regurgitation (TR) trends. The Kaplan-Meier method was used to estimate cumulative incidences of CIED complications and ≥moderate grade TR. 61 patients were included; mean age was 44.0±17.8 years and 32 (52.4%) were female. 30 (49.2%) transvenous and 31 (50.8%) epicardial CIEDs; 44 (72.1%) had pacemaker and 17 (27.9%) had implantable cardioverter defibrillators. The most common indications were heart block (39.3%) and sudden cardiac death secondary prevention (27.9%). 29 (47.5%) patients received new CIED implantation (22 epicardial, 7 transvenous) at TV surgery. 20 (32.8%) patients had preexisting CIED (14 transvenous, 6 epicardial) at time of TV surgery; among those with transvenous CIEDs, 8 had lead exteriorization to the TV, 3 had extraction followed by epicardial CIED implantation, 2 had preexisting coronary sinus leads, and 1 had transmyocardial lead placement. 12 (19.7%) patients underwent CIED implantation (9 transvenous, 3 epicardial) without concurrent surgery. The most frequent CIED complications were high pacing threshold (n=4) and infection (n=3). The 5 year cumulative incidence of CIED complications was 27.6% with no significant difference between epicardial and transvenous CIEDs (31.1% vs. 25.9%, p=0.96). Having a lead traverse the TV was associated with increased risk of having ≥moderate grade TR at 5 years (57.1% vs. 5.3%, p=0.021). Stability of lead parameters including sensing and threshold was seen for both epicardial and transvenous leads. In patients with EA, epicardial and transvenous CIEDs have similar lead performance and safety, but a lead crossing the TV was associated with worse TR. Epicardial CIEDs should be considered at the time of TV surgery in patients with EA and CIED indication.

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