Transcatheter Leadless Pacing in Children: A PACES Collaborative Study in the Real-World Setting.

Background: Transcatheter Leadless Pacemakers (TLP) are a safe and effective option for adults with pacing indications. These devices may be an alternative in pediatric patients and patients with congenital heart disease for whom repeated sternotomies, thoracotomies, or transvenous systems are unfavorable. However, exemption of children from clinical trials has created uncertainty over the indications, efficacy, and safety of TLP in the pediatric population.

Electrophysiologic Management and Considerations for D-transposition of the Great Arteries After Atrial Baffle Repair.

This unique case highlights the electrophysiologic management and risk assessment of sudden cardiac death in a 35-year-old woman with a history of D-transposition of the great arteries status post-Mustard atrial switch repair.

Management and outcomes of atrial fibrillation in 241 healthy children and young adults: Revisiting "lone" atrial fibrillation-A multi-institutional PACES collaborative study.

Background: Atrial fibrillation (AF) in healthy children and young adults is rare. Risk of recurrence and treatment efficacy are not well defined.

Objective: The purpose of this study was to assess recurrence patterns and treatment efficacy in AF.

Pulmonary Cusp Positioning of a Right Ventricular Outflow Tract Ventricular Tachycardia in a Pediatric Patient Identified Using Intracardiac Echocardiography.

Ventricular premature beats originating from the right ventricular outflow tract can have myocardial extensions to the pulmonary valve and pulmonary artery. Treatment may consist of catheter ablation combined with the use of three-dimensional mapping to determine the exact location of ectopy. The location of ectopy relative to the pulmonary valve may be hard to ascertain.

Desmoplakin Cardiomyopathy, a Fibrotic and Inflammatory Form of Cardiomyopathy Distinct From Typical Dilated or Arrhythmogenic Right Ventricular Cardiomyopathy.

Background: Mutations in desmoplakin (), the primary force transducer between cardiac desmosomes and intermediate filaments, cause an arrhythmogenic form of cardiomyopathy that has been variably associated with arrhythmogenic right ventricular cardiomyopathy. Clinical correlates of cardiomyopathy have been limited to small case series.

Methods: Clinical and genetic data were collected on 107 patients with pathogenic mutations and 81 patients with pathogenic plakophilin 2 () mutations as a comparison cohort.

Exercise-induced syncope and Brugada syndrome.

Brugada syndrome (BrS) is a hereditary condition that is characterized by ST elevation, ventricular tachycardia or fibrillation, and sudden cardiac death in otherwise healthy patients. Life-threatening arrhythmias generally occur, while at rest, with fever or during vagotonic states. Exercise is generally not considered a trigger for ventricular arrhythmias or syncope in patients with BrS.

Noninvasive predictors of perioperative atrial arrhythmias in patients with tetralogy of Fallot undergoing pulmonary valve replacement.

Background: Patients with tetralogy of Fallot (TOF) have increased risk of atrial arrhythmias.

Hypothesis: A measure of atrial dispersion, the P-wave vector magnitude (Pvm), can identify patients at risk for perioperative atrial flutter (AFL) or intra-atrial re-entrant tachycardia (IART) in a large TOF cohort.

Methods: We performed a blinded, retrospective analysis of 158 TOF patients undergoing pulmonary valve replacement between 1997 and 2015.

Noninvasive Predictors of Ventricular Arrhythmias in Patients With Tetralogy of Fallot Undergoing Pulmonary Valve Replacement.

Objectives: This study sought to test the hypothesis that a vectorcardiographic parameter, the QRS vector magnitude (QRSVm), can risk stratify those patients at risk for sustained spontaneous ventricular arrhythmias (VAs) or ventricular arrhythmia inducibility (VAI) in a large cohort of patients with tetralogy of Fallot (TOF).

Background: Patients with TOF have an increased risk of VAs, but predicting those at risk can often be challenging.

Methods: Blinded retrospective analyses of 177 TOF patients undergoing pulmonary valve replacement (PVR) between 1997 and 2015 were performed.

Lower spatial QRS-T angle rules out sustained ventricular arrhythmias in children with hypertrophic cardiomyopathy.

Unlabelled: Introduction The spatial peaks QRS-T angle accurately distinguishes children with hypertrophic cardiomyopathy from their healthy counterparts. The spatial peaks QRS-T angle is also useful in risk stratification for ventricular arrhythmias. We hypothesised that the spatial peaks QRS-T angle would be useful for the prediction of ventricular arrhythmias in hypertrophic cardiomyopathy patients under 23 years of age.

Repolarization Vector Magnitude Differentiates Kawasaki Disease from Normal Children.

Introduction: Kawasaki disease (KD) is the leading cause of acquired heart disease in children. The 12-lead electrocardiogram (ECG) changes in patients during the acute phase of KD include flattened T waves and prolonged corrected QT intervals (QTc). We set out to determine the 12-lead ECG and vectorcardiography predictors for identification of patients with KD and which of these predictors would be clinically useful for early identification of those with coronary artery anomalies (CAA).

The spatial QRS-T angle outperforms the Italian and Seattle ECG-based criteria for detection of hypertrophic cardiomyopathy in pediatric patients.

Introduction: The spatial peaks QRS-T angle has been shown to differentiate adult patients with hypertrophic cardiomyopathy (HCM) from controls. We hypothesized that the spatial peaks QRS-T angle would, in isolation, be more accurate than the Italian 12-lead ECG Pre-participation Screening criteria or the Seattle criteria for detecting hypertrophic cardiomyopathy (HCM) in pediatric patients.

Methods: A retrospective study of pediatric patients with HCM compared to age and gender-matched control patients was undertaken.

Intermittent versus Persistent Wolff-Parkinson-White Syndrome in Children: Electrophysiologic Properties and Clinical Outcomes.

Background: Intermittent Wolff-Parkinson-White (WPW) syndrome is considered to have a lower risk of sudden death. Fewer data exist regarding electrophysiologic (EP) characteristics and the natural history of intermittent WPW in children.

Methods: All patients with WPW age 1-18 years at a single institution (1996-2013) were reviewed.

Borderline and Long QT Syndrome in Adolescent Athletes Taking Medications.

Purpose: Adolescent athletes undergo preparticipation screening for long QT syndrome. Medications that are associated with QT interval prolongation are increasingly being prescribed to precollege students. The side effect profile of these medications during exercise is unknown.

Vectorcardiographic predictors of ventricular arrhythmia inducibility in patients with tetralogy of Fallot.

Introduction: Vectorcardiography (VCG) may have predictive value in Tetralogy of Fallot (TOF) patients undergoing ventricular arrhythmia inducibility (VAI) electrophysiology studies (EPs).

Materials And Methods: Blinded, retrospective analyses of 37 adult TOF patients undergoing EPs prior to pulmonary valve replacements were performed (21 female, median age 37years). VAI was evaluated from EPs and resting 12-lead electrocardiograms, respectively using QRS and heart rate adjusted Q-T intervals, spatial QRS-T angles (peaks), T-wave and QRS-wave (QRSwave vc) component vector root mean squares.

Predictors of myocardial recovery in pediatric tachycardia-induced cardiomyopathy.

Background: Tachycardia-induced cardiomyopathy (TIC) carries significant risk of morbidity and mortality, although full recovery is possible. Little is known about the myocardial recovery pattern.

Objective: The purpose of this study was to determine the time course and predictors of myocardial recovery in pediatric TIC.

Tachyarrhythmia following Norwood operation: a single-center experience.

Background: The purpose of this study was to characterize tachyarrhythmias in children following the Norwood procedure.

Methods: This is a single-center retrospective study including all children who underwent stage I Norwood procedure (n = 98; January 2003-September 2011). The primary outcome measure is the development of tachyarrhythmia during hospitalization after the Norwood procedure.

Tachyarrhythmias after the Norwood procedure: relationship and effect of vasoactive agents.

Tachyarrhythmias are common after the Norwood stage I palliation. The effects of vasoactive medications on the development of post-operative tachyarrhythmias have not been studied. The primary objective was to identify associations between postoperative tachyarrhythmias and vasoactive medication usage after Norwood stage 1 palliation.

The fate of nontargeted endocardial leads during the extraction of one or more targeted leads in pediatrics and congenital heart disease.

Introduction: Extraction of targeted endocardial leads can lead to inadvertent damage or dislodgement of nontargeted leads, though the frequency of this has not been reported in pediatrics and congenital heart disease (CHD).

Methods: This is a retrospective review of lead extraction procedures from 2002 to 2010 from two pediatric and CHD centers evaluating unplanned interventions on nontargeted leads during the extraction of one or more targeted leads.

Results: Nontargeted leads were present in the 22 lead extraction procedures performed on 20 patients with median age 15 years (interquartile range, 11.

Laser lead extraction in congenital heart disease: a case-controlled study.

Introduction: Patients with congenital heart disease (CHD) and implanted cardiac rhythm management devices have a high rate of endocardial lead issues requiring lead extraction. Laser lead extraction (LLE) is a promising modality for lead management though few studies have evaluated this technique in CHD.

Methods: This is a retrospective, case-controlled (gender- and age-matched, without CHD) analysis of all CHD patients from 2002-2010 at a single institution who underwent LLE as a primary extraction modality.