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Clinically significant bradycardia is an uncommon problem in children, but one that can cause significant morbidity and sometimes necessitates implantation of a pacemaker. The most common causes of bradycardia are complete heart block (CHB), which can be congenital or acquired, and sinus node dysfunction, which is rare in children with structurally normal hearts. Pacemaker is indicated as therapy for the majority of children with CHB, and while early mortality is lower in postnatally diagnosed CHB than in fetal CHB, it is still up to 16%. In young children, less invasive transvenous pacemaker systems can be technically challenging to place and carry a high risk of complications, often necessitating surgical epicardial pacemaker placement, which usually entails a median sternotomy. We report three cases of pediatric patients referred for pacemaker implantation for different types of bradycardia, treated at our institution with oral albuterol with therapeutic results that avoided the need for surgical pacemaker implantation at that time., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objectives: We aimed to measure the frequency and factors associated with anodal stimulation in a pediatric population with epicardial pacing leads., Background: In bipolar pacemakers, capture of the myocardium typically occurs at the cathode. However, AS with capture at the anode has been described. This has not been described in epicardial pacemakers., Methods: Retrospective data were collected from patients ≤ 21 years of age with permanent bipolar epicardial ventricular pacemakers from 1/2017 to 1/2018. AS was defined as a clear change on surface ECG in at least one of the 12 leads assessed by two blinded pediatric electrophysiologists., Results: Twenty-four bipolar leads in 23 patients were included in the study. One patient had both biventricular leads tested. Median age was 7.1 years (IQR 5.0-10.9), weight was 20.9 kg (IQR 16.5-33.5), and 65% were male. Testing was performed at a median of 2.8 years (IQR 1.6-6.1) after implant. Congenital heart disease was present in 57%. Complete heart block was the pacemaker indication in 78%. AS was identified in 16/24 (67%) of leads tested. Identification of AS was associated with presence of congenital heart disease (p = 0.004) and 3DD between electrodes (p = 0.04)., Conclusions: AS is common in pediatric patients and was associated with a history of congenital heart disease and greater estimated 3DD between electrodes. The prevalent nature of AS may allow clinicians to utilize existing pacemakers as multisite pacing systems., (© 2021 Wiley Periodicals LLC.)

Prolongation of the QT interval and development of torsades de pointes are known in patients with complete heart block and profound bradycardia. We report the case of a patient with complete heart block and torsades, with long QT seen during a period of junctional tachycardia at a rate faster than the minimum pacemaker rate.

Background: Three-dimensional electroanatomic mapping systems (3D mapping) allow the creation of an anatomic representation of the cardiac anatomy and real-time monitoring of transvenous pacing catheters. Their use to facilitate pacemaker (PM) and implantable cardioverter defibrillator (ICD) lead placement in the pediatric population has not been well defined. We sought to review our experience using 3D mapping as an adjunct to fluoroscopy for positioning of permanent PM and ICD leads in pediatric patients., Methods: We performed a retrospective review of all patients ≤21 years of age who underwent PM or ICD implantation with the aid of the Ensite Velocity system (Ensite) from May 2012 to September 2014. Fluoroscopy was utilized in addition to 3D mapping to aid obtaining vascular access, to visualize fixation of the lead to the myocardium, and to evaluate lead length within the vasculature., Results: Nineteen patients (mean age 14.6 ± 4.4 years) underwent placement of active fixation PM leads (N = 17) and/or ICD leads (N = 10) with the aid of Ensite. All leads were successfully placed (eight atrial, 19 ventricular). Fluoroscopic exposure was a mean of 3.2 minutes (± 2.8, 0.1-10.5). There was one lead dislodgement., Conclusions: Ensite was used successfully as an adjunct to fluoroscopy in all patients in whom it was attempted to aid the placement of permanent transvenous PM and ICD leads. Future evaluation could focus on the use of 3D mapping systems to further minimize or eliminate fluoroscopy from PM and ICD implantations., (©2015 Wiley Periodicals, Inc.)

Background: Transtelephonic monitoring (TTM) of pediatric patients with cardiac pacemakers (PMs) has been shown to have high sensitivity and specificity in identifying PM malfunction. The objective of this study is to determine if there is a difference in the rate of abnormal TTM findings in transvenous versus epicardial PM systems., Methods: Our TTM database was reviewed. Patients younger than 21 years of age enrolled for TTM between 1990 and 2010 were included. The abnormal TTM recordings (not including elective replacement indicator) were identified. Logistic regression was used for statistical analysis. Note that P < 0.05 was considered significant., Results: We identified 186 patients. There were 75 (40%) epicardial systems. The mean age at TTM enrollment was 6.8 ± 5.9 years (2 months-20.2 years). There were 41 (22%) patients with abnormal TTM findings. The abnormalities were found in 23/75 (31%) epicardial and 18/111 (16%) transvenous systems (odds ratio [OR]: 2.3, 95% confidence interval [CI]: 1.13-4.62, P = 0.02). When controlling for age and presence of heart disease the OR for abnormal transmission in epicardial systems compared with transvenous was 2.1 (95% CI: 1.03-4.43, P = 0.04). Patients with epicardial systems were more likely to have capture abnormalities on TTM than transvenous systems (OR: 6.1, 95% CI: 1.9-19.5, P = 0.002)., Conclusion: Pediatric patients with epicardial PM systems are more likely to have abnormal TTM test (particularly capture problems) than patients with transvenous systems regardless of age or presence of heart disease. Consequently, patients with epicardial PM systems should be followed closely with TTM., (©2013, The Authors. Journal compilation ©2013 Wiley Periodicals, Inc.)

Objective: Although many Fontan patients undergo pacemaker placement, there are few studies characterizing this population. Our purpose was to compare clinical characteristics, functional status and measures of ventricular performance in Fontan patients with and without a pacemaker., Patients and Design: The National Heart, Lung, and Blood Institute funded Pediatric Heart Network Fontan Cross-Sectional Study characterized 546 Fontan survivors. Clinical characteristics, medical history and study outcomes (Child Health Questionnaire [CHQ]), echocardiographic evaluation of ventricular function, and exercise testing) were compared between subjects with and without pacemakers., Results: Of 71 subjects with pacemakers (13%), 43/71 (61%) were in a paced rhythm at the time of study enrollment (age 11.9 ± 3.4 years). Pacemaker subjects were older at study enrollment, more likely to have single left ventricles, and taking more medications. There were no differences in age at Fontan or Fontan type between the pacemaker and no pacemaker groups. There were no differences in exercise performance between groups. CHQ physical summary scores were lower in the pacemaker subjects (39.7 ± 14.3 vs. 46.1 ± 11.2, P =.001). Ventricular ejection fraction z-score was also lower (-1.4 ± 1.9 vs. -0.8 ± 2.0, P =.05) in pacemaker subjects., Conclusions: In our cohort of Fontan survivors, those with a pacemaker have poorer functional status and evidence of decreased ventricular systolic function compared to Fontan survivors without a pacemaker., (© 2012 Wiley Periodicals, Inc.)

Purpose: Lead failure is a common complication of pacemaker placement in the pediatric population and is often due to insulation breaks or lead fracture. Usually these leads are replaced with new leads. This study describes the surgical technique of lead repair and investigates the longevity of such repairs., Methods: Retrospective review of lead repairs performed in the pediatric population (<21 years old) between December 1995 and December 2008. Operative records, pre-operative interrogation data, and most recent follow-up data were reviewed., Results: Twenty-one lead repairs were performed on 17 patients. Sixteen were ventricular leads and five were atrial leads. There were 14 insulation defect repairs, six new lead tip splicings, and one lead extender splicing. The repaired leads were followed for a mean time of 3.6 ± 3.0 years (range, 0.3-10.6 years). The freedom from repaired lead failure by Kaplan-Meier survival analysis was 89% at 5 years and 22% at 10 years. One atrial lead and two ventricular leads have been replaced since the repairs. One atrial lead has had a repeat insulation break repair since the initial repair. One of the five atrial leads had an elevated threshold at follow-up. Five of 16 ventricular leads had elevated thresholds at follow-up. All leads had normal impedance at follow-up., Conclusion: The option of pacemaker lead repair, if surgically feasible, offers an alternative to lead replacement in the pediatric population and may extend the longevity of pacemaker leads in this population.