Inappropriate implantable cardioverter-defibrillator shocks in repaired tetralogy of fallot patients: Prevalence and electrophysiological mechanisms

Int J Cardiol Heart Vasc. 2020 May 26;28:100543. doi: 10.1016/j.ijcha.2020.100543. eCollection 2020 Jun.


Background: Patients with Tetralogy of Fallot (TOF) are at increased risk for sudden cardiac death, often undergo implantable cardioverter defibrillator (ICD) implantation at younger ages, and are at greater risk of experiencing inappropriate shocks. We investigated occurrences of ICD shocks in TOF patients to identify prevalence, characteristics associated with inappropriate shocks, and therapeutic interventions after inappropriate shocks.

Methods: Records of patients with repaired TOF and ICD implantation who were followed at Columbia University Irving Medical Center between 1/1/2000 and 5/1/2019 were analyzed.

Results: 44 patients with repaired TOF and ICD implantation were reviewed. Mean age at implantation was 39 ± 13 years. Eight (18%) patients received both appropriate and inappropriate shocks, 6 (14%) received only appropriate shocks, and 3 (7%) received only inappropriate shocks. Three patients received inappropriate shocks for sinus tachycardia, 7 for atrial arrhythmias, and 1 for noise artifact. Inappropriately shocked patients had lower beat per minute (bpm) cutoff values for ICD therapy (mean = 162 ± 24 bpm vs. 182 ± 16 bpm, p = 0.007). After inappropriate shocks, 1 patient underwent lead replacement, 1 had the VT cutoff increased, and 6 were treated with medications.

Conclusions: One quarter of TOF patients with ICDs experienced inappropriate shock therapy, the timing of which was most often clustered within the first two years after implant or years later. Lower shock therapy zones were associated with increased risk for inappropriate shocks, and the majority of inappropriate shocks resulted from atrial arrhythmias with rapid ventricular response. Treatments for inappropriate shocks included increasing VT therapy bpm and rhythm and/or rate control medications.

Keywords: Congenital heart disease; Implantable cardioverter-defibrillator; Inappropriate shock therapy; Tetralogy of fallot.