High-density "APLE" Mapping-Activation, Propagation, Low Voltage, and Electrogram Evaluation with the HD Grid for Atrioventricular Nodal Re-entry Tachycardia Ablation.

This is the first case series to evaluate high-density mapping of the triangle of Koch (TOK) using the HD Grid to guide slow-pathway ablation integrating activation, propagation (with wave collision), low-voltage signals, and atrial electrogram appearance. We will describe our technique and the results in this case series. Using three-dimensional mapping and the HD Grid, patients underwent high-density voltage mapping of the TOK. Ablation site selection was based on properties during sinus rhythm with late activation, at or above the propagation wave collision, over low voltage, and with appropriate electrogram appearance. Five patients underwent mapping of the slow pathway using the HD Grid. Their median age was 14 years, their median weight was 54.1 kg, and their median height was 161.5 cm. The TOK was mapped with the HD Grid for a median of 3 min. The procedure was successful in all patients using this technique. The median lesion number to the site of success was 3, with a median total number of cryotherapy lesions of 11. No radiation was used. There were no recurrences. Using activation, propagation wave, low voltage, and electrogram appearance when mapping for slow-pathway localization and ablation with the HD Grid can be successful, results in high-density maps, and is relatively faster.

Epicardial Implantation of a Micra™ Pacemaker in a Premature Neonate with Congenital Complete Heart Block.

Pacemaker implantation in neonates can be challenging due to their small size. Even pulse generators adapted for pediatric patients, such as the Microny device (Abbott, Chicago, IL, USA), are proportionately large in comparison to the size of the smallest newborns. Due to anatomic considerations, such as small vascular and ventricular sizes, leadless pacemakers and transvenous implantation in the youngest neonatal population remain unsuitable. Even so, the desire for leadless devices has prompted the industry to create the smallest pacemakers available. Adapting the smaller Micra™ transcatheter leadless pacing system (Medtronic, Minneapolis, MN, USA) for an epicardial pacing application may be advantageous to the smallest patients. This case illustrates the use of a Micra™ device modified with a header block to serve as the pulse generator in a ventricular epicardial pacing system for a 1-day-old, 2.68-kg patient with complete heart block.

Improvements in Accuracy and Confidence in Rhythm Identification After Cardiac Surgery Using the AtriAmp Signals.

Objectives: Following cardiac surgery, 50% to 60% of patients with congenital heart disease will experience an arrhythmia. These arrhythmias are associated with increased morbidity and mortality. Therefore, rapid and accurate identification is paramount to the improvement of patient outcome. We hypothesize that the AtriAmp, a device which allows atrial electrogram (AEG) display on the bedside monitors, will increase provider accuracy and confidence in arrhythmia diagnosis. Design: A prospective observational study. Electrograms were collected from post-operative patients from the bedside monitors surface ECG and an AEG using the AtriAmp. A 12-question online survey was given to critical care and cardiology providers at 9 different programs across the country as well as being posted to the AAP SOCC fall newsletter. Six questions displayed signals from only the surface leads, while the other 6 showed the same arrhythmias with an AEG obtained from the AtriAmp. Answers were then evaluated for confidence and accuracy. A paired t-test along with mixed method modeling was used to assess the data. Setting: Cardiac pediatric ICU. Subjects: Providers in pediatric cardiology and pediatric critical care were evaluated on their ability to diagnose arrhythmias on surface ECG and AEG obtained from bedside monitor. Interventions: The accuracy and confidence of diagnosis of both surface and AEG signals were evaluated through an on-line survey. Results: Eighty-eight providers completed the survey. The study showed that interpreting with the AtriAmp signal, compared to the surface ECG only, significantly increased the accuracy (P = .002) and confidence in provider rhythm diagnosis (P < .001). Junctional ectopic tachycardia, sinus tachycardia, and complete heart block had the most significant increase in accuracy of diagnose when using the AtriAmp signal (P < .001, P = .002, P = .010, respectively). Conclusion: Use of the AtriAmp increased provider accuracy and confidence in post-operative rhythm diagnosis when compared to diagnosis using the surface electrograms only.

IV Sotalol Use in Pediatric and Congenital Heart Patients: A Multicenter Registry Study.

Background There is limited information regarding the clinical use and effectiveness of IV sotalol in pediatric patients and patients with congenital heart disease, including those with severe myocardial dysfunction. A multicenter registry study was designed to evaluate the safety, efficacy, and dosing of IV sotalol. Methods and Results A total of 85 patients (age 1 day-36 years) received IV sotalol, of whom 45 (53%) had additional congenital cardiac diagnoses and 4 (5%) were greater than 18 years of age. In 79 patients (93%), IV sotalol was used to treat supraventricular tachycardia and 4 (5%) received it to treat ventricular arrhythmias. Severely decreased cardiac function by echocardiography was seen before IV sotalol in 7 (9%). The average dose was 1 mg/kg (range 0.5-1.8 mg/kg/dose) over a median of 60 minutes (range 30-300 minutes). Successful arrhythmia termination occurred in 31 patients (49%, 95% CI [37%-62%]) with improvement in rhythm control defined as rate reduction permitting overdrive pacing in an additional 18 patients (30%, 95% CI [19%-41%]). Eleven patients (16%) had significant QTc prolongation to >465 milliseconds after the infusion, with 3 (4%) to >500 milliseconds. There were 2 patients (2%) for whom the infusion was terminated early. Conclusions IV sotalol was safe and effective for termination or improvement of tachyarrhythmias in 79% of pediatric patients and patients with congenital heart disease, including those with severely depressed cardiac function. The most common dose, for both acute and maintenance dosing, was 1 mg/kg over ~60 minutes with rare serious complications.

Atrial Electrography for Postoperative Tachyarrhythmia Analysis in Patients.

The over 400,000 cardiac surgeries performed in the United States each year hold a risk for the postoperative complication of arrhythmias. Currently, bedside monitoring of surface electrocardiogram leads is used to interpret arrhythmias despite the evidence that atrial electrograms (AEGs) offer superior rhythm discrimination. This hesitancy to use the AEG may be due to a lack of training for practitioners in interpreting AEGs; therefore, our goal was to create an algorithm for the diagnosis of tachyarrhythmia using an AEG that can be utilized by any health care practitioner. Our algorithm classifies the most prevalent type of tachyarrhythmias following cardiac surgery. To allow rhythm identification, we categorized them based on their atrial to ventricular signal ratio, which is uniquely apparent on AEGs. Other considerations were given to rhythm regularity, consistency, P-wave axis, and rate. The algorithm includes the most common postoperative arrhythmias differentiated based on a unique branch-point approach, which walks through the steps in arrhythmia discrimination. Both rendered and collected AEGs are included as references for further understanding and interpretation of tachyarrhythmias. The utility of AEGs for rhythm discrimination post-cardiac surgery is established and recent technology can provide real-time and continuous monitoring; however, practitioner training may be inadequate. To bridge this divide, we created an algorithm so that existing atrial wires can be better used for an enhanced rhythm interpretation via AEGs.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

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. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients: Executive summary.

Guidelines for the implantation of cardiac implantable electronic devices (CIEDs) have evolved since publication of the initial ACC/AHA pacemaker guidelines in 1984 [1]. CIEDs have evolved to include novel forms of cardiac pacing, the development of implantable cardioverter defibrillators (ICDs) and the introduction of devices for long term monitoring of heart rhythm and other physiologic parameters. In view of the increasing complexity of both devices and patients, practice guidelines, by necessity, have become increasingly specific. In 2018, the ACC/AHA/HRS published Guidelines on the Evaluation and Management of Patients with Bradycardia and Cardiac Conduction Delay [2], which were specific recommendations for patients >18 years of age. This age-specific threshold was established in view of the differing indications for CIEDs in young patients as well as size-specific technology factors. Therefore, the following document was developed to update and further delineate indications for the use and management of CIEDs in pediatric patients, defined as ≤21 years of age, with recognition that there is often overlap in the care of patents between 18 and 21 years of age. This document is an abbreviated expert consensus statement (ECS) intended to focus primarily on the indications for CIEDs in the setting of specific disease/diagnostic categories. This document will also provide guidance regarding the management of lead systems and follow-up evaluation for pediatric patients with CIEDs. The recommendations are presented in an abbreviated modular format, with each section including the complete table of recommendations along with a brief synopsis of supportive text and select references to provide some context for the recommendations. This document is not intended to provide an exhaustive discussion of the basis for each of the recommendations, which are further addressed in the comprehensive PACES-CIED document [3], with further data easily accessible in electronic searches or textbooks.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

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. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

Outcomes of Pediatric Patients With Defibrillators Following Initial Presentation With Sudden Cardiac Arrest.

BACKGROUND: Implantable cardioverter defibrillators (ICD) are recommended for secondary prevention after sudden cardiac arrest (SCA). The outcomes of pediatric patients receiving an ICD after SCA remain unclear. The objective of this study is to evaluate outcomes, future risk for appropriate shocks, and identify characteristics associated with appropriate ICD therapy during follow-up. METHODS: Multicenter retrospective analysis of patients (age ≤21 years) without prior cardiac disease who received an ICD following SCA. Patient/device characteristics, cardiac function, and underlying diagnoses were collected, along with SCA event characteristics. Patient outcomes including complications and device therapies were analyzed. RESULTS: In total, 106 patients were included, median age 14.7 years. Twenty (19%) received appropriate shocks and 16 (15%) received inappropriate shocks (median follow-up 3 years). First-degree relative with SCA was associated with appropriate shocks (P<0.05). In total, 40% patients were considered idiopathic. Channelopathy was the most frequent late diagnosis not made at time of presentation. Neither underlying diagnosis nor idiopathic status was associated with increased incidence of appropriate shock. Monomorphic ventricular tachycardia (hazard ratio, 4.6 [1.2-17.3]) and family history of sudden death (hazard ratio, 6.5 [1.4-29.8]) were associated with freedom from appropriate shock in a multivariable model (area under the receiver operating characteristic curve, 0.8). Time from diagnoses to evaluation demonstrated a nonlinear association with freedom from appropriate shock (P=0.015). In patients >2 years from implantation, younger age (P=0.02) and positive exercise test (P=0.04) were associated with appropriate shock. CONCLUSIONS: The risk of future device therapy is high in pediatric patients receiving an ICD after SCA, irrelevant of underlying disease. Lack of a definitive diagnosis after SCA was not associated with lower risk of subsequent events and does not obviate the need for secondary prophylaxis.

Cardiovascular Collapse with Intravenous Amiodarone in Children: A Multi-Center Retrospective Cohort Study.

OBJECTIVE: To determine the incidence of cardiovascular collapse in children receiving intravenous (IV) amiodarone and to identify the population at risk. DESIGN: A multicenter study of patients ≤ 18 years of age who received intravenous amiodarone between January 2005 and December 2015. A retrospective analysis was performed to identify patients who developed cardiovascular collapse (bradycardia and/or hypotension). RESULTS: Of 456 patients who received amiodarone, cardiovascular collapse occurred in 47 patients (10%). Patient risk factors for collapse in a univariate analysis were as follows: age < 3 months (p = 0.04), depressed cardiac function (p < 0.001), blood pressure below 3rd percentile (p < 0.001), high lactate at baseline (p < 0.001). Administration risk factors included bolus administration (p = 0.04), and bolus administration over ≤ 20 min (p = 0.04). In multivariate analysis, age, baseline blood pressure less than 3rd percentile, and rapid bolus delivery were independent risk factors for cardiovascular collapse in the study group. The mortality rate was significantly higher in the collapse group (28% versus 8%). CONCLUSION: We found an association between IV amiodarone administration and the risk of developing cardiovascular collapse in a significant subset of children. Extreme caution and careful hemodynamic monitoring is recommended when using IV amiodarone in this population, especially in young infants, hemodynamically compromised patients, and in patients receiving rapid amiodarone bolus administration.

A Minimally Invasive Approach for Placing Sew-On Epicardial Leads in the Child.

Traditionally, pacing leads are placed transvenously, although smaller pediatric patients who require permanent pacemakers may benefit from delaying tranvenous lead placement until they are larger. Alternative, minimally invasive atrioventricular pacing options have not previously existed for this patient population, leaving many of these children with large sternotomies or thoracotomies. Using three port sites and an adjustable shaft dual-needle suturing device, we placed a steroid-eluting, sew-on epicardial lead on the right atrium of a 9-year-old patient. This is one of the earliest reported cases of a minimally invasive technique for sew-on epicardial lead placement on the atrium of a child. Although based on a single case, we believe that this approach is safe, reliable, and reproducible and that it can be used to place leads on any aspect of the heart. Adoption of this technique will allow for earlier atrioventricular pacing, which may decrease the incidence of pacemaker syndrome, and thus improve overall outcomes in this patient population.

Outcomes During Intended Fluoroscopy-free Ablation in Adults and Children.

Electroanatomic mapping (EAM) systems facilitate the elimination of fluoroscopy during electrophysiologic (EP) studies and ablations. The rate and predictors of fluoroscopy requirements while attempting fluoroscopy-free (FF) ablations are unclear. This study aimed (1) to investigate the rates of fluoroscopic use and acute success in patients initially referred for FF ablation and (2) to identify procedural characteristics associated with fluoroscopic use in patients in whom FF ablation was initially planned (IFF). We performed a retrospective review of all patients who underwent IFF EP study or ablation between 2010 and 2013. Patient and procedural characteristics were compared between those with successful FF procedures and those who subsequently required fluoroscopy during their procedure. An FF EP study with or without ablation was performed in 124 patients during 138 procedures for either supraventricular or idiopathic ventricular arrhythmias. Of the 138 procedures, 105 of them were performed without fluoroscopy. In the remaining 33 cases, fluoroscopy was used for an average of 1.21 minutes ± 1.18 minutes. Acute procedural success was achieved in 97% of both FF and fluoroscopy procedures. The primary reason for fluoroscopy use was as a guide for transseptal puncture. There were no significant differences between FF and fluoroscopy procedures with respect to catheter placement time or complication rate. In conclusion, in this single-center study of IFF procedures, despite careful case selection for IFF ablation, 24% of IFF cases ultimately required minimal fluoroscopy. Fluoroscopy and FF procedures had similar rates of procedural success and complications. Additional large prospective studies are required to further investigate the safety and efficacy of FF ablations.

Neonatal complex arrhythmias possibly related to a TTN mutation.

We describe a neonate born with complex arrhythmias that included concurrent atrial and ventricular tachycardias. Genetic testing demonstrated a mutation in the TTN gene, which codes for titin, a large protein found in striated muscle sarcomeres. The complex arrhythmias were successfully treated with amiodarone and flecainide. The patient remains asymptomatic with normal biventricular function. We speculate that the complex arrhythmias and TTN gene mutation may be related.