Desmoplakin Cardiomyopathy in Pediatric Patients: A Distinct, Underrecognized Cohort of Arrhythmogenic Cardiomyopathy.

Background: cardiomyopathy is a distinct subset of arrhythmogenic cardiomyopathy, reported primarily in adults, that has predominantly left ventricular involvement and features of myocarditis. Clinical characteristics, risk stratification, and management of pediatric patients with variants are not well known. We sought to identify phenotypic features and prognosis of pediatric patients with pathogenic or likely pathogenic variants.

Validation of a Demography-Based Adaptive QTc Formula using Pediatric and Adult Datasets Acquired from Humans and Guinea Pigs.

Introduction: A variety of QT rate-correction (QTc) formulae have been utilized for both clinical and research purposes. However, these formulae are not universally effective, likely due to significant influences of demographic diversity on the QT-HR relationship. To address this limitation, we proposed an adaptive QTc (QTcAd) formula that adjusts to subject demographics (i.

Vigorous Exercise in Patients With Congenital Long QT Syndrome: Results of the Prospective, Observational, Multinational LIVE-LQTS Study.

Background: Whether vigorous exercise increases risk of ventricular arrhythmias for individuals diagnosed and treated for congenital long QT syndrome (LQTS) remains unknown.

Methods: The National Institutes of Health-funded LIVE-LQTS study (Lifestyle and Exercise in the Long QT Syndrome) prospectively enrolled individuals 8 to 60 years of age with phenotypic and/or genotypic LQTS from 37 sites in 5 countries from May 2015 to February 2019. Participants (or parents) answered physical activity and clinical events surveys every 6 months for 3 years with follow-up completed in February 2022.

Emerging Technologies for the Smallest Patients.

Pediatric and congenital heart disease patients may require cardiac implantable electronic device implantation, inclusive of pacemaker, ICD, and implantable cardiac monitor, for a variety of etiologies. While leads, generators, and monitors have decreased in size over the years, they remain less ideal for the smallest patients. The potential for a miniature pacemaker, fetal micropacemaker, improving leadless technology, and rechargeable devices creates hope that the development of pediatric-focused devices will increase.

Analysis of risk stratification and prevention of sudden death in pediatric patients with hypertrophic cardiomyopathy: Dilemmas and clarity.

Hypertrophic cardiomyopathy (HCM) has been considered the most common cause of sudden death (SD) in the young. However, introduction of implantable cardioverter-defibrillators (ICDs) in HCM has proved highly effective and the mainstay of preventing SD in children, adolescents, and adults by terminating malignant ventricular tachyarrhythmias. Nevertheless, ICD decision making is generally regarded as more difficult in pediatrics, and the strategy for selecting ICD patients from this population remains without consensus.

In-vivo evaluation of catheter integrity with the use of a novel catheter torque tool.

Introduction: Electrophysiology studies and ablation procedures require strength, steadiness, and dexterity to manipulate catheters. We have previously described a novel catheter torque tool (Peritorq) that improves torqueability and stability and decreases user muscle fatigue. The objective was to evaluate measures of catheter integrity with and without the torque tool in place using multiple diagnostic and ablation catheters in an adult porcine model.

Cardiac evaluation of hospitalized children with 2019 coronavirus (COVID-19) infection at a single large quaternary center.

Background: Cardiac complications of serious SARS-CoV-2 infections, especially Multisystem Inflammatory Syndrome of Children (MIS-C) are well described, however current studies have not considered pediatric patients hospitalized with no cardiac concerns. We established a protocol for cardiac evaluation of all admitted COVID-19 patients three weeks post-discharge, irrespective of cardiac concerns. We assessed cardiovascular outcomes and hypothesized that patients with absent cardiac concerns are at lower risk for cardiac abnormalities.

Percutaneous epicardial pacing in infants using direct visualization: A feasibility animal study.

Background: Pacemaker implantation in infants and small children is limited to epicardial lead placement via open chest surgery. We propose a minimally invasive solution using a novel percutaneous access kit.

Objective: To evaluate the acute safety and feasibility of a novel percutaneous pericardial access tool kit to implant pacemaker leads on the epicardium under direct visualization.

A novel videoscope and tool kit for percutaneous pericardial access under direct visualization.

Background: Pericardial access is necessary for the application of epicardial cardiac therapies including ablation catheters, pacing and defibrillation leads, and left atrial appendage closure systems. Pericardial access under fluoroscopic guidance is difficult in patients without pericardial effusions and may result in coronary artery damage, ventricular injury, or perforation with potentially life-threatening pericardial bleeding in up to 10% of cases. There is a clinical need for a pericardial access technique to safely deliver epicardial cardiac therapies.

Design and Functionality of a Multilumen Thoracic Access Port for Pericardial Access Under Direct Visualization.

Small vasculature, venous obstruction, or congenital anomalies can preclude transvenous access to the heart, often resulting in open chest surgery to implant cardiac therapy leads for pacing, defibrillation, or cardiac resynchronization. A minimally invasive approach under direct visualization could reduce tissue damage, minimize pain, shorten recovery time, and obviate the need for fluoroscopy. Therefore, PeriPath was designed as a single-use, low-cost pericardial access tool based on clinical requirements.

Pediatric Electrophysiology Device Needs: A Survey from the Pediatric and Congenital Electrophysiology Society Taskforce on Pediatric-Specific Devices.

Background There are few US Food and Drug Administration (FDA)-approved devices specifically aimed at the pediatric patient with arrhythmia. This has led to a high off-label utilization of devices in this vulnerable population. The Pediatric and Congenital Electrophysiology Society (PACES), the international organization representing pediatric and congenital heart disease arrhythmia specialists, developed a task force to comprehensively address device development issues relevant to pediatric patients with congenital arrhythmia.

Muscle usage and workload assessment of cardiac ablation procedure with the use of a novel catheter torque tool in a pediatric simulator.

Background: Cardiac ablation catheters are small in diameter and pose ergonomic challenges that can affect catheter stability. Significant finger dexterity and strength are necessary to maneuver them safely. We evaluated a novel torque tool to reduce muscle activation when manipulating catheters and improve perceived workload of ablation tasks.

An infant phantom for pediatric pericardial access and electrophysiology training.

Background: Cardiac procedures in infants and children require a high level of skill and dexterity owing to small stature and anatomy. Lower incidence of procedure volume in this population results in fewer clinical opportunities for learning. Simulators have grown in popularity for education and training, though most existing simulators are often cost-prohibitive or model adult anatomy.

2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Developed in collaboration with and endorsed by the Heart Rhythm Society (HRS), the American College of Cardiology (ACC), the American Heart Association (AHA), and the Association for European Paediatric and Congenital Cardiology (AEPC) Endorsed by the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS).

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences.