Limited Relationship Between Echocardiographic Measures and Electrocardiographic Markers of Left Ventricular Size in Healthy Children.

Pediatric ECG standards have been defined without echocardiographic confirmation of normal anatomy. The Pediatric Heart Network Normal Echocardiogram Z-score Project provides a racially diverse group of healthy children with normal echocardiograms. We hypothesized that ECG and echocardiographic measures of left ventricular (LV) dimensions are sufficiently correlated in healthy children to imply a clinically meaningful relationship. This was a secondary analysis of a previously described cohort including 2170 digital ECGs. The relationship between 6 ECG measures associated with LV size were analyzed with LV Mass (LVMass-z) and left ventricular end-diastolic volume (LVEDV-z) along with 11 additional parameters. Pearson or Spearman correlations were calculated for the 78 ECG-echocardiographic pairs with regression analyses assessing the variance in ECG measures explained by variation in LV dimensions and demographic variables. ECG/echocardiographic measurement correlations were significant and concordant in 41/78 (53%), though many were significant and discordant (13/78). Of the 6 ECG parameters, 5 correlated in the clinically predicted direction for LV Mass-z and LVEDV-z. Even when statistically significant, correlations were weak (0.05-0.24). R2 was higher for demographic variables than for echocardiographic measures or body surface area in all pairs, but remained weak (R2 ≤ 0.17). In a large cohort of healthy children, there was a positive association between echocardiographic measures of LV size and ECG measures of LVH. These correlations were weak and dependent on factors other than echocardiographic or patient derived variables. Thus, our data support deemphasizing the use of solitary, traditional measurement-based ECG markers traditionally thought to be characteristic of LVH as standalone indications for further cardiac evaluation of LVH in children and adolescents.

Current State of Cardiac Implantable Electronic Device Remote Monitoring in Pediatrics and Congenital Heart Disease: A PACES-Sponsored Quality Improvement Initiative.

Cardiac implantable electronic device (CIED) remote transmissions are an integral part of longitudinal follow-up in pediatric and adult congenital heart disease (ACHD) patients. To evaluate baseline CIED remote monitoring (RM) data among pediatric and ACHD centers prior to implementation of a Pediatric and Congenital Electrophysiology  Society (PACES)-sponsored quality improvement (QI) project. This is a cross-sectional study of baseline CIED RM. Centers self-reported baseline data: individual center RM compliance was defined as high if there was > 80% achievement and low if < 50%. A total of 22 pediatric centers in the USA and Australia submitted baseline data. Non-physicians were responsible for management of the RM program in most centers: registered nurse (36%), advanced practice provider (27%), combination (23%), and third party (9%). Fifteen centers (68%) reported that > 80% of their CIED patients are enrolled in RM and only two centers reported < 50% participation. 36% reported high compliance of device transmission within 14 days of implant and 77% of centers reported high compliance of CIED patients enrolled in RM. The number of centers achieving high compliance differed by device type: 36% for pacemakers, 50% for ICDs, and 55% for Implantable Cardiac Monitors (ICM). All centers reported at least 50% adherence to recommended follow-up for PM and ICD, with 23% low compliance rate for ICMs. Based on this cross-sectional survey of pediatric and ACHD centers, compliance with CIED RM is sub-optimal. The PACES-sponsored QI initiative will provide resources and support to participating centers and repeat data will be evaluated after PDSA cycles.

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. Further, alternative approaches that avoid the need for a transvenous or surgical approach may add more options to the toolbox for the pediatric and congenital electrophysiologist.

Diagnostic yield of ambulatory cardiac monitoring in pediatric patients with palpitations.

Background: Palpitations are a frequent reason for referral to pediatric cardiology providers and diagnostic workup includes ambulatory cardiac monitoring. While common practice, the diagnostic yield is unknown in the pediatric population. The objective is to evaluate the diagnostic yield of 24-h Holter and extended ambulatory cardiac monitoring in pediatric patients with palpitations. Methods and Results: All pediatric patients aged 10-18 years who had ambulatory cardiac monitoring (1-30 days) through the Pocket Electrocardiogram (PocketECG™) system (Medi-Lynx) between January 2016 and July 2020 were included. Patients with an International Classification of Diseases-10 diagnosis code of palpitations (R00.2) during enrollment were evaluated separately. Tachyarrhythmia diagnoses included atrial fibrillation (AF), nonsustained supraventricular tachycardia (nSVT), supraventricular tachycardia (SVT), nonsustained ventricular tachycardia (nVT), and ventricular tachycardia (VT). Age, heart rates, arrhythmia type, and symptomatic transmission data were collected and analyzed. A total of 2388 patients (mean age 11.6 years, 58% F) with the R00.2 code had ambulatory cardiac monitoring (28% 24-h Holter, 72% extended) performed during the study period and there were 6287 total patients (mean age 13.9 years, 54% F) that underwent ambulatory cardiac monitoring (42% 24-h Holter, 58% extended) during that time. Of 2388 patients, 321 (13%) were diagnosed with tachyarrhythmia: AF (9), nSVT (192), SVT (59), and nVT (61). In the overall cohort, 764 (12%) patients were diagnosed with tachyarrhythmia: AF (22), nSVT (478), SVT (85), nVT (177), and VT (2). Symptomatic transmissions with normal cardiac rhythm were common in the R00.2 (n = 1697, 71%) and overall (n = 3848, 61%) groups. No episodes of nSVT, SVT, nVT, or VT were associated with symptomatic transmissions. Conclusion: Ambulatory cardiac monitors are an integral part of the diagnostic workup for pediatric palpitations patients and have demonstrated a high yield of combined positive arrhythmia diagnoses and symptomatic normal transmissions. Further prospective study of this population with the integration of clinical information is warranted.

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. METHODS: Diagnostic and ablation catheters were inserted through the femoral or jugular vein into areas of the right atrium, coronary sinus (CS), and right ventricle. Electrical measurements including impedance, sensing, and capture thresholds were obtained with and without the torque tool. Ablation lesions (30 s) were given at different locations using both irrigated and nonirrigated catheters and measurements were recorded with and without the torque tool. RESULTS: Procedures were performed in eight adult pigs. Measurements with and without the torque tool in all locations did not differ significantly using any of the catheters. With the nonirrigated ablation catheter there was a significant difference in maximum (mean 1.7 W, p = .03) and average power (mean 9.1 W, p = .04) delivery at the PS tricuspid valve, but there were no other differences with the irrigated or nonirrigated catheters. Subjective assessment by the operator revealed a substantial improvement in maneuverability, ability to transfer torque, and stability within the cardiac space. CONCLUSION: In an in-vivo environment, a novel catheter torque tool subjectively improved catheter manipulation and did not have a significant impact on the integrity of electrophysiologic catheters. Further study including additional catheters and in-vivo human testing is indicated.

Genetic screening of relatives of decedents experiencing sudden unexpected death: medical examiner's office referrals to a multi-disciplinary cardiogenetics program.

Currently, no standardized system exists for evaluating and testing at-risk family members of decedents with abnormal post-mortem genetic testing in cases of sudden unexpected death (SUD). The goal of this study was to evaluate the outcomes of referrals made by an urban medical examiner's office to a multi-disciplinary cardiogenetics clinic. Relatives of decedents with pathogenic/likely pathogenic (P/LP) variants or variants of unknown significance (VUS) in genes known to be associated with cardiomyopathies and/or arrhythmias were identified by the New York City Office of Chief Medical Examiner and referred to the Cardiogenetics Clinic at Montefiore Medical Center. Familial referrals of 15 decedents (median 15 years, range 2 days to 57 years) were evaluated. Variants in 13 genes were identified among decedents (9 arrhythmia, 5 cardiomyopathy). P/LP variants were identified in both arrhythmia (RYR2, SCN5A) and cardiomyopathy syndrome (MYBPC3 (2), MYH7) genes. Thirty-two family members were referred, and 14 variants were detected. One pathogenic (MYBPC3) and two likely pathogenic (RYR2, MYH7) mutations were identified. Referral of at-risk family members of decedents who experienced SUD based on informative post-mortem genetic testing for cardiac and genetic evaluation is warranted, as family studies help to reclassify variants and prevent additional sudden death.

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. The objective was to evaluate measurable success, user perception of workload, and muscle usage when completing a simulated ablation task with and without the use of a catheter torque tool. METHODS: Cardiology attendings and fellows were fitted with surface electromyographic (EMG) sensors on 6 key muscle groups in the left hand and forearm. A standard ablation catheter was inserted into a pediatric cardiac ablation simulator and subjects navigated the catheter tip to 6 specific electrophysiologic targets, including a 1-min simulated radiofrequency ablation lesion. Time to complete the task, number of attempts required to complete the lesion, and EMG activity normalized to percentage of maximum voluntary contraction were collected throughout the task. The task was completed 4 times, twice with and twice without the torque tool, in semi-randomized order. A NASA Task Load Index survey was completed by the participant at the conclusion of each task. RESULTS: Time to complete the task and number of attempts to create a lesion were not altered by the tool. Subjectively, participants reported a significant decrease in physical demand, effort, and frustration, and a significant increase in performance. Muscle activation was decreased in 4 of 6 muscle groups. CONCLUSION: The catheter torque tool may improve the perceived workload of cardiac ablation procedures and reduce muscle fatigue caused by manipulating catheters. This may result in improved catheter stability and increased procedural safety.

Ambulatory Cardiac Monitoring in Infants with Supraventricular Tachycardia.

Supraventricular tachycardia (SVT) is a frequent cause of tachyarrhythmia in infants < 1 year of age and ambulatory cardiac monitoring is an important tool for diagnosis and follow-up of these patients. We retrospectively reviewed 594 infants (mean age 4.05 months, SD 3.55; 54% M) who underwent ambulatory cardiac monitoring (69% 24 h Holter, 31% extended monitor) through the Pocket ECG system (MediLynx) between January 2016 and July 2020. 170 patients who had the ICD-10 code I47.1 for SVT used at enrollment were analyzed separately. 49 (8.3%) patients had sustained SVT or non-sustained SVT (nSVT) during the study period, including 20 patients (11.8%) who had the ICD-10 code I47.1 at enrollment. Extended ambulatory cardiac monitors detected 61% of all patients with nSVT or SVT and was superior when compared to 24 h Holter (p < 0.0001). In the overall group, the first episode of SVT or nSVT was detected within 24 h of monitoring in 40/49 patients (82%). 48/49 patients (98%) were diagnosed within a week of monitoring and the single remaining patient was diagnosed with nSVT at day 15 of monitoring. There was no significant difference in minimal, maximal, and average heart rate between patients with and without ICD-10 code I47.1 at enrollment or between patients with and without SVT or nSVT. Despite their low yield, ambulatory cardiac monitors are an important diagnostic tool. The ideal length of monitoring in patients with known or suspected SVT has yet to be defined, although all patients in our cohort were identified by day 15 of monitoring.

A paediatric cardiology handbook for the digital age.

INTRODUCTION: Paediatric cardiology fellows, tasked with studying a large and dynamic field, may benefit from a quick-access digital resource that reflects contemporary practice. The objective of this study was to evaluate the effectiveness of a paediatric cardiology handbook smartphone app in enhancing the accessibility of information such as guidelines and recommendations for paediatric cardiology fellows. MATERIALS AND METHODS: The Peds Cardiology Handbook app (iOS) was designed using XCode and Swift programming. A pre-app survey and download instructions were sent to the US paediatric cardiology fellows, followed by a post-app survey. Fellows were asked to rate the ease of rapid access to various types of information. Responses were compared pre- and post-app using McNemar's test and Wilcoxon signed-rank test. RESULTS: Two-hundred and thirty paediatric cardiology fellows were contacted; 23% (n = 53) completed both the pre- and post-app surveys and were included for analysis. After using the app, fellows found it easier to quickly access information in eight out of nine domains evaluated (p < 0.05). All fellows found the app easy to use (100%), most felt that the app was well-organised (98%), contained reliable information (92%), and was useful for finding quick answers to clinical questions (87%). DISCUSSION: The Peds Cardiology Handbook iPhone app is a useful, reliable tool that provides quick access to high-yield information, including guidelines and references. Overall, paediatric cardiology fellows found it easier to rapidly access clinically relevant information after using the app. Future studies may be necessary to evaluate long-term use and impact on evidence-based practice.

Utility of Exercise Stress Testing in Pediatric Patients with T-Wave Inversions.

T-wave inversions (TWI) in inferolateral electrocardiogram (ECG) leads (II, III, aVF, V5-V6) are often suggestive of cardiac pathology in adult patients. They are a common reason of additional testing in pediatric patients. The goal of the study is to determine correlation of exercise stress test T-wave response with diagnosis of cardiac pathology. This is a retrospective review of pediatric patients < 21 years with the finding of baseline TWI in inferior (II, III, avF) and/or lateral (V5-V6) leads on 12-lead ECG. All patients underwent treadmill exercise stress test (EST) and an echocardiogram within 1 year of each other. Demographics, baseline ECG findings, echocardiogram results, and EST data were recorded. T-wave reversion was considered complete if T waves demonstrated normalization during exercise, partial if there was minimal improvement, and no response if there was no change or worsening of inversions. In our cohort of 72 patients with a mean age 14.6 (± 2.9) years and 61% males, 59 (82%) had a structurally normal heart. Thirteen patients had evidence of structural or functional heart disease. Of the 59 patients, 83% had either complete or partial T-wave response. Among the 13 patients with heart disease, two patients had genetic testing consistent with risk for hypertrophic cardiomyopathy and had complete and partial response on EST. Exercise stress testing for TWI in pediatric patients has low sensitivity and specificity for the diagnosis of cardiac disease and routine use in this patient population may not be indicated.

QTc Prolongation in Pediatric Patients with Diabetic Ketoacidosis.

OBJECTIVE: To investigate the association between diabetic ketoacidosis (DKA) and prolonged QTc interval and to assess for correlation between DKA severity and QTc prolongation. STUDY DESIGN: Retrospective observational study in a pediatric hospital. Patients admitted with DKA diagnosed by laboratory criteria and an electrocardiogram (ECG) performed during a period of acidosis were identified using Looking Glass Clinical Analytics. Data including age, sex, pH, electrolytes, anion gap, and ECG variables were collected. Patients were excluded if they had a prior diagnosis of prolonged QTc or were taking QTc prolonging medications. Severity of DKA was classified as mild (pH 7.24-7.3), moderate (pH 7-7.24), or severe (pH <7). ECGs were read by a pediatric electrophysiologist and QTc interval was manually calculated utilizing the Bazett formula. RESULTS: Ninety-six patients were included (mean age 15.2 ± 4.2 years, pH 7.12 ± 0.12, bicarbonate 8.6 ± 3.7 mmol/L, potassium 5.3 ± 1.1 mEq/L). Mean QTc interval for all patients in DKA was 454 ± 32 msec. Mean QTc in the mild group was 441 ± 22 msec, moderate group 460 ± 36 msec, and severe group 461 ± 34 msec. There was a significant difference in QTc interval across DKA severity groups (P = .05). There was a significant association between higher anion gaps and greater QTc intervals (r = 0.21, P = .04). CONCLUSIONS: Thirty-one percent of pediatric patients with DKA demonstrated QTc prolongation on ECG. Severity of DKA and worsening acidosis were associated with increased prolongation of the QTc. Further study is required to evaluate the clinical impact of these findings.

Multisystem Inflammatory Syndrome in Children: An International Survey.

OBJECTIVES: To describe presentation, hospital course, and predictors of bad outcome in multisystem inflammatory syndrome in children (MIS-C). METHODS: Retrospective data review of a case series of children meeting the published definition for MIS-C who were discharged or died between March 1, 2020, and June 15, 2020, from 33 participating European, Asian, and American hospitals. Data were collected through a Web-based survey and included clinical, laboratory, electrocardiographic, and echocardiographic findings and treatment management. RESULTS: We included 183 patients with MIS-C: male sex, 109 (59.6%); mean age 7.0 ± 4.7 years; Black race, 56 (30.6%); obesity, 48 (26.2%). Overall, 114 of 183 (62.3%) had evidence of severe acute respiratory syndrome coronavirus 2 infection. All presented with fever, 117 of 183 (63.9%) with gastrointestinal symptoms, and 79 of 183 (43.2%) with shock, which was associated with Black race, higher inflammation, and imaging abnormalities. Twenty-seven patients (14.7%) fulfilled criteria for Kawasaki disease. These patients were younger and had no shock and fewer gastrointestinal, cardiorespiratory, and neurologic symptoms. The remaining 77 patients (49.3%) had mainly fever and inflammation. Inotropic support, mechanical ventilation, and extracorporeal membrane oxygenation were indicated in 72 (39.3%), 43 (23.5%), and 4 (2.2%) patients, respectively. A shorter duration of symptoms before admission was found to be associated with poor patient outcome and for extracorporeal membrane oxygenation and/or death, with 72.3% (95% confidence interval: 0.56-0.90; P = .006) increased risk per day reduction and 63.3% (95% confidence interval: 0.47-0.82; P < .0001) increased risk per day reduction respectively. CONCLUSIONS: In this case series, children with MIS-C presented with a wide clinical spectrum, including Kawasaki disease-like, life-threatening shock and milder forms with mainly fever and inflammation. A shorter duration of symptoms before admission was associated with a worse outcome.

Cardiac Abnormalities Seen in Pediatric Patients During the SARS-CoV2 Pandemic: An International Experience.

Background During the SARS-CoV2 pandemic, there has been increase in hyperinflammatory presentation in previously healthy children with a variety of cardiac manifestations. Our objective is to describe the cardiac manifestations found in an international cohort of 55 pediatric cases with multi-system inflammatory syndrome (MIS-C) during the SARS-CoV2 pandemic. Methods and Results We reviewed data on previously healthy pediatric patients (≤18 years) with structurally normal hearts who presented at hospitals in the United States, United Kingdom, Spain and Pakistan with MIS-C and had consultation with a pediatric cardiologist. Data collected included demographics, clinical presentation, laboratory values, electrocardiographic abnormalities, echocardiographic findings and initial therapies. A total of 55 patients presented with MIS-C. Thirty-five patients (64%) had evidence of decreased left ventricular function, 17 (31%) had valvulitis, 12 (22%) with pericardial effusion and 11 (20%) with coronary abnormalities. Twenty-seven (49%) required ICU admission and 24 (44%) had evidence of shock. Eleven patients (20%) fulfilled complete Kawasaki disease criteria and had lower NT pro-BNP, D-dimer and ferritin levels compared with those who did not fulfill criteria. Electrophysiologic abnormalities occurred in 6 patients and included complete atrioventricular (AV) block, transient AV block and ventricular tachycardia. Conclusions We describe the first international cohort of pediatric patients with MIS-C during the SARS-CoV2 pandemic with a range of cardiac manifestations. This paper brings awareness and alertness to the global medical community to recognize these children during the pandemic and understand the need for early cardiology evaluation and follow-up.

Surgical pericardial adhesions do not preclude minimally invasive epicardial pacemaker lead placement in an infant porcine model.

BACKGROUND: Pericardial adhesions in infants and small children following cardiac surgery can impede access to the epicardium. We previously described minimally invasive epicardial lead placement under direct visualization in an infant porcine model using a single subxiphoid incision. The objective of this study was to assess the acute feasibility of this approach in the presence of postoperative pericardial adhesions. METHODS: Adhesion group piglets underwent left thoracotomy with pericardiotomy followed by a recovery period to develop pericardial adhesions. Control group piglets did not undergo surgery. Both groups underwent minimally invasive epicardial lead placement using a 2-channel access port (PeriPath) inserted through a 1 cm subxiphoid incision. Under direct thoracoscopic visualization, pericardial access was obtained with a 7-French sheath, and a pacing lead was affixed against the ventricular epicardium. Sensed R-wave amplitudes, lead impedances and capture thresholds were measured. RESULTS: Eight piglets underwent successful pericardiectomy and developed adhesions after a median recovery time of 45 days. Epicardial lead placement was successful in adhesion (9.5 ± 2.7 kg, n = 8) and control (5.6 ± 1.5 kg, n = 7) piglets. There were no acute complications. There were no significant differences in capture thresholds or sensing between groups. Procedure times in the adhesion group were longer than in controls, and while lead impedances were significantly higher in the adhesion group, all were within normal range. CONCLUSIONS: Pericardial adhesions do not preclude minimally invasive placement of epicardial leads in an infant porcine model. This minimally invasive approach could potentially be applied to pediatric patients with prior cardiac surgery.

Speckle Tracking Echocardiography in Pediatric Patients with Premature Ventricular Contractions.

Premature ventricular contractions (PVCs) in pediatric patients without structural heart disease and normal left ventricular systolic function rarely require therapy, though it is unknown whether these patients have subclinical cardiac dysfunction. Speckle tracking echocardiography is an additional means of evaluating cardiac function in asymptomatic pediatric PVC patients with normal standard measures of left ventricular (LV) function. Asymptomatic pediatric patients (< 21 years) without congenital heart disease, LV ejection fraction (LVEF) ≥ 55% and PVC burden ≥ 5% on 24-h Holter monitor were included. Demographic information, exercise stress test results, standard echocardiographic measures of LV systolic function and PVC morphology by 12-lead ECG were collected. Peak global systolic longitudinal strain (GLS) from apical four-chamber view was analyzed offline. 29 patients were identified (mean age 11.7 ± 5.8 years, 49.2 ± 25.3 kg, 59% male). Mean PVC burden was 12.0 ± 7.0% (range 5-37.5%). 14/29 (48%) had exercise stress testing with evidence of PVCs; 9/14 (64%) had PVC suppression at a mean heart rate (HR) of 160 ± 23 bpm and 5/14 (36%) did not suppress at a mean maximum HR of 188 ± 9 bpm. All patients had normal strain values by speckle tracking echocardiography (mean LV GLS - 22.5 ± 2.0%, LV global circumferential strain - 25.3 ± 3.9 and RV GLS - 24.1 ± 3.0%). There was no correlation between PVC burden and cardiac function parameters. Asymptomatic pediatric patients without structural heart disease, preserved LVEF/shortening fraction and PVC burden ≥ 5% demonstrated normal cardiac function including strain patterns indicating no evidence of subclinical cardiac dysfunction. Larger scale studies and longitudinal evaluation of left ventricular function using speckle tracking echocardiography is warranted in this population.