Immediate and Intermediate-Term Effects of Disease Mitigation Measures on Activity Levels as Measured by Implantable Cardiac Devices.

Routine activities of daily living are known to effect overall wellbeing, cardiovascular health, and quality of life. A decrease in daily activity level is recognized as a contributing factor to increasing rates of obesity and a decline in overall health. This study sought to evaluate the effect of disease mitigation strategies on activity level by evaluating changes in recorded cardiac parameters as measured by implantable cardiac devices for a period of time prior to and following implementation of COVID-19 mitigation efforts in Arizona.

Thoracoscopic Cardiac Sympathetic Denervation: Adjunct Therapy for Secondary Prevention of Life-Threatening Ventricular Arrhythmias in Children.

Background: Cardiac sympathetic denervation (CSD) is a surgical option for patients with life-threatening ventricular arrhythmias. Previously described cohorts included populations in which CSD was performed for primary and secondary prevention. We report the efficacy of CSD as adjunct therapy in children with medically refractory life-threatening arrhythmias.

A Clinical Risk Score to Improve the Diagnosis of Tachycardia-Induced Cardiomyopathy in Childhood.

Tachycardia-induced cardiomyopathy (TIC) is a treatable cause of heart failure in children, but there is little information as to which clinical variables best discriminate TIC from other forms of cardiomyopathy. TIC cases with dilated cardiomyopathy (DC) from 16 participating centers were identified and compared with controls with other forms of DC. Presenting clinical, echocardiographic, and electrocardiographic characteristics were collected.

Catheter ablation of supraventricular tachyarrhythmia after extracardiac Fontan surgery.

Background: Extracardiac total cavopulmonary connection (E-TCPC) is widely performed for single ventricle palliation, yet there is little experience with catheter ablation in this population.

Objectives: We hypothesized that atrial tachycardia substrates after primary E-TCPC would be similar to those in other forms of congenital heart disease and that catheter ablation could be performed effectively using a primarily transconduit approach.

Methods: Catheter ablation characteristics of patients with E-TCPC from 9 centers were collected.

Spectrum and Prevalence of CALM1-, CALM2-, and CALM3-Encoded Calmodulin Variants in Long QT Syndrome and Functional Characterization of a Novel Long QT Syndrome-Associated Calmodulin Missense Variant, E141G.

Background: Calmodulin (CaM) is encoded by 3 genes, CALM1, CALM2, and CALM3, all of which harbor pathogenic variants linked to long QT syndrome (LQTS) with early and severe expressivity. These LQTS-causative variants reduce CaM affinity to Ca(2+) and alter the properties of the cardiac L-type calcium channel (CaV1.2).

Management of pediatric tachyarrhythmias on mechanical support.

Background: Pediatric patients with persistent arrhythmias may require mechanical cardiopulmonary support. We sought to classify the population, spectrum, and success of current treatment strategies.

Methods And Results: A multicenter retrospective chart review was undertaken at 11 sites.

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.

The Multicenter Pediatric and Adult Congenital EP Quality (MAP-IT) Initiative-rationale and design: report from the pediatric and congenital electrophysiology society's MAP-IT taskforce.

Multicenter clinical registries are increasingly recognized as valuable tools for establishing benchmarks, facilitating patient-centered quality improvement and research. In 2010, the Pediatric and Congenital Electrophysiology Society convened a taskforce of its members to design, construct, and implement a clinical registry known as the Multicenter Pediatric and Adult Congenital EP Quality (MAP-IT) Initiative. The present aim of the MAP-IT Initiative is to create an infrastructure by which we can measurably improve patient-centered outcomes and reduce complications associated with electrophysiology studies and catheter ablation in pediatric and congenital heart disease patients.

Unusually high association of hypertrophic cardiomyopathy and complex heart defects in children with fasciculoventricular pathways.

Introduction: Fasciculoventricular pathways (FVPs) are rare causes of preexcitation that do not mediate tachycardias. We report a two-center experience of pediatric patients with FVP and an unexpectedly high association of complex congenital heart defects (CHDs), chromosomal anomalies, and hypertrophic cardiomyopathy.

Methods: A retrospective review of the electrophysiology database at two institutions was performed to identify patients with FVP from January 2000 to January 2011.

Limited utility of exercise-stress testing to prevent T-wave oversensing in pediatric internal cardioverter defibrillator recipients.

Background: Inappropriate internal cardioverter defibrillator (ICD) therapies may result from T-wave oversensing (TWOS) during exertion in children. The aim of this study was to evaluate the utility of an exercise treadmill test to predict inappropriate ICD therapies secondary to TWOS.

Methods: Eligible pediatric ICD recipients underwent exercise-stress testing with concomitant evaluation of all intracardiac electrograms.

An unusual resolution of T-wave oversensing in an implantable cardioverter defibrillator in a child with long QT syndrome.

T-wave oversensing poses a clinical challenge often resulting in inappropriate therapies and shocks. T-wave oversensing can often be resolved by certain programmable adjustments while accepting a small risk of undersensing ventricular fibrillation. The following case depicts a girl with LQT whose transvenous defibrillator repeatedly demonstrated T-wave oversensing despite optimizing various programmable features and a separate pace/sense lead.

Impact of a computer assisted navigation system on radiation exposure during pediatric ablation procedures.

Background: During catheter ablation procedures, non-radiologic navigation systems may reduce fluoroscopic exposure and energy applications, as well as improve procedural success rates.

Objective: To examine the impact of a non-radiologic navigation system on ablation procedures in pediatric patients, the procedural characteristics and success rates prior to and following incorporation of the LocaLisa (LL) navigation system into a pediatric electrophysiology laboratory were compared.

Methods: Between January 2000 and April 2005, 246 consecutive patients underwent catheter ablation for either Atrioventricular Reentry Tachycardia AVRT (168) or Atrioventricular Nodal Reentry Tachycardia AVNRT (78).

Transcatheter cryotherapy for the treatment of supraventricular tachyarrhythmias in children: a single center experience.

Introduction: Transcatheter cryotherapy is an emerging technology for the treatment of children with supraventricular tachyarrhythmias. Limited data exist regarding the use of cryoablation therapy in children. We report a single center's experience with transcatheter cryoablation in the pediatric population.