What have we learned in the last 20 years? A comparison of a modern era pediatric and congenital catheter ablation registry to previous pediatric ablation registries.

Background: Since the onset of pediatric catheter ablation, the pediatric electrophysiology community has reported outcomes via various registries (PAPCA [Prospective Assessment After Pediatric Cardiac Ablation], PCAR [Pediatric Catheter Ablation Registry]). Most recently, a modern era pediatric and congenital ablation registry (MAP-IT [Multicenter Pediatric and Congenital EP Quality Initiative]) was developed for eventual incorporation into the National Cardiovascular Data Registry (NCDR) IMPACT (Improving Pediatric and Adult Congenital Treatment) registry.

Objective: The purpose of this study was to describe initial findings from the MAP-IT pilot registry and to compare these findings to earlier registries.

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.

The incidence of chromosome abnormalities in neonates with structural heart disease.

Objectives: This study was conducted to determine the prevalence of chromosomal anomalies in newborns with structural heart disease admitted to the cardiac intensive care unit (CICU) at Nicklaus Children's Hospital (NCH).

Methods: A retrospective review identified newborns age 30 days or less admitted to NCH CICU between 2004 and 2010. Patients with structural heart disease who required admission to our CICU and received karyotype or karyotype and fluorescent in situ hybridization (FISH) testing were included in the study.

Use of smartphone technology in cardiology.

Smartphone-based technologies along with broadband connectivity are changing the way modern cardiology is practiced. The ever broadening connectivity and increasing capabilities of smartphone-based technologies can better monitor, diagnose, and prevent cardiovascular diseases. Researchers can leverage the ubiquitous use of smartphone-based technologies and their constant stream of biometric data to establish large community-based clinical research studies.

SPEAR Trial: Smartphone Pediatric ElectrocARdiogram Trial.

Objectives: Smartphone-enabled ECG devices have the potential to improve patient care by enabling remote ECG assessment of patients with potential and diagnosed arrhythmias. This prospective study aimed to assess the usefulness of pediatric ECG tracings generated by the AliveCor device (Oklahoma City, OK) and to assess user satisfaction.

Study Design: Enrolled pediatric patients with documented paroxysmal arrhythmia used the AliveCor device over a yearlong study period.

Pediatric electrocardiographic imaging applications.

Noninvasive electrocardiographic imaging (ECGI) has been used in pediatric and congenital heart patients to better understand their electrophysiologic substrates. In this article we focus on the 4 subjects related to pediatric ECGI: (1) ECGI in patients with congenital heart disease and Wolff–Parkinson–White syndrome, (2) ECGI in patients with hypertrophic cardiomyopathy and preexcitation, (3) ECGI in pediatric patients with Wolff–Parkinson–White syndrome, and (4) ECGI for pediatric cardiac resynchronization therapy.

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.

Assessment of intrathoracic impedance algorithm in the pediatric and adult congenital population.

Background: Decreased intrathoracic impedance has been used in adults to predict heart failure (HF) exacerbations. A commercial algorithm, OptiVol® (Medtronic Inc., Minneapolis, MN, USA), identifies patients with decreased impedance.

Duplication of 20p12.3 associated with familial Wolff-Parkinson-White syndrome.

Wolff-Parkinson-White (WPW) syndrome is caused by preexcitation of the ventricular myocardium via an accessory pathway which increases the risk for paroxysmal supraventricular tachycardia. The condition is often sporadic and of unknown etiology in the majority of cases. Autosomal dominant inheritance and association with congenital heart defects or ventricular hypertrophy were described.

Updates on the inherited cardiac ion channelopathies: from cell to clinical.

The inherited channelopathies are a rare, heterogeneous group of diseases with widely variable clinical presentations and courses. Systematic clinical and experimental work has led to identification of disease-causing genetic mutations and their biophysical manifestation. The process by which the knowledge base is developed, from genetic mutation, to cardiac myocyte, to whole heart, and finally to clinical presentation, has dramatically expanded our understanding of these diseases.

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.

Electrophysiologic substrate and intraventricular left ventricular dyssynchrony in nonischemic heart failure patients undergoing cardiac resynchronization therapy.

Background: Electrocardiographic imaging (ECGI) is a method for noninvasive epicardial electrophysiologic mapping. ECGI previously has been used to characterize the electrophysiologic substrate and electrical synchrony in a very heterogeneous group of patients with varying degrees of coronary disease and ischemic cardiomyopathy.

Objective: The purpose of this study was to characterize the left ventricular electrophysiologic substrate and electrical dyssynchrony using ECGI in a homogeneous group of nonischemic cardiomyopathy patients who were previously implanted with a cardiac resynchronization therapy (CRT) device.

Outcomes of heart transplantation using donor hearts from infants with sudden infant death syndrome.

Background: Uncertainty exists whether hearts from infants who have died of sudden infant death syndrome (SIDS) are acceptable for transplantation because the mechanism of death in SIDS remains unclear. We analyzed post-transplant outcomes in infants who received a heart from a donor where SIDS was the primary cause of brain death.

Methods: This retrospective multicenter cohort study used data from the Organ Procurement and Transplant Network (OPTN).

Use of a wearable automated defibrillator in children compared to young adults.

Background: A wearable automated external defibrillator has been shown to be efficacious in the prevention of sudden death in adults who had a history of cardiac arrest but who did not have a permanent internal cardioverter/defibrillator (ICD) placed. The use of a wearable defibrillator has not been established in the pediatric population.

Methods: We retrospectively reviewed the clinical database for the wearable external defibrillator from ZOLL Lifecor Corporation (Pittsburgh, PA, USA).

Cryoablation with an 8-mm tip catheter for pediatric atrioventricular nodal reentrant tachycardia is safe and efficacious with a low incidence of recurrence.

Background: Cryoablation with 4- and 6-mm tip ablation catheters has been demonstrated to be safe and effective in the treatment of atrioventricular nodal reentrant tachycardia (AVNRT) in pediatric patients, albeit with a higher rate of clinical recurrence. Limited information is available regarding efficacy, mid-term outcomes, and complications related to the use of the 8-mm Freezor Max Cryoablation catheter (Medtronic, Minneapolis, MN, USA) in pediatric patients.

Methods: We performed a retrospective review of all pediatric patients with normal cardiac anatomy who underwent an ablation procedure for treatment of AVNRT using the 8-mm tip Cryoablation catheter at three large pediatric academic arrhythmia centers.

Current management of pediatric dilated cardiomyopathy.

Purpose Of Review: American and European guidelines for treatment of adult heart failure have been recently revised. This review will reconcile those guidelines to recent studies and experience in the treatment of pediatric dilated cardiomyopathy.

Recent Findings: Therapy for pediatric dilated cardiomyopathy includes establishing a diagnosis for diagnostic-specific therapies as well as preventive strategies for anthracycline toxicity and muscular dystrophy.

Management of postoperative pediatric cardiac arrhythmias: current state of the art.

Postoperative arrhythmias in the pediatric population remain a significant source of morbidity and mortality despite advances in surgical techniques. Although our understanding of these arrhythmias has improved, the number of therapeutic options to treat them has not increased significantly in recent years. However, in the specific case of bradyarrhythmias, the use of pacemakers has reduced morbidity and mortality significantly.

Cardiac resynchronization therapy in pediatric congenital heart disease: insights from noninvasive electrocardiographic imaging.

Background: Electrocardiographic imaging (ECGI) is a novel electrophysiologic imaging modality that may help guide patient selection and lead placement for cardiac resynchronization therapy (CRT).

Objective: The purpose of this study was to apply noninvasive ECGI to pediatric heart failure patients with congenital heart disease (CHD) undergoing evaluation for CRT.

Methods: ECGI was applied in eight patients with CHD who were either being evaluated for CRT or undergoing CRT.