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Background: Atrial fibrillation and atrial flutter are relatively rare in young people and the incidence of thromboembolic complications is unknown. These issues contribute to the limited utility of present guidelines regarding anticoagulation in this population., Objective: To report the number of thromboembolic complications in a cohort of pediatric and young adult patients presenting with atrial fibrillation (AFib) or atrial flutter (AFl) while also assessing anticoagulation practice in a multicenter cohort of young patients with these arrhythmias., Methods: Multicenter, retrospective cohort of patients aged < 25 years old who presented with atrial flutter (AFl) or atrial fibrillation (AFib) between 2000 and 2019 to several large, quaternary pediatric centers, excluding episodes occurring within 30 days of an invasive cardiac procedure., Results: There were 311 episodes of AFib/AFl among 210 patients with a median age of 17 (IQR 14, 20) years. Structural heart disease (SHD) was present in 120 patients (57%) and 20 patients (10%) had a primary cardiomyopathy. Twelve AFib/AFl episodes (8 with AFL, 4 with AFib) were associated with a thrombus, of which 8 (67%) patients had SHD, 2 (17%) had cardiomyopathy and 5 (42%) ventricular dysfunction. The CHADSVASc score was 0-3 for all patients with a thrombus. Of patients with no thrombus at presentation who were cardioverted, 102 (34%) patients were discharged on aspirin and 80 (27%) on anticoagulation. Two patients had symptoms concerning for a thromboembolic event on follow-up, but none had a newly documented thrombus., Conclusions: Thromboembolic complications occur in children and young adults presenting with AFib/AFl. The majority are associated with SHD, cardiomyopathy, and/or ventricular dysfunction. CHADSVASc was calculated for each patient and was of limited utility. Thromboembolic complications at follow up after cardioversion are rare, and anticoagulation strategies at discharge varied., (© 2024 Wiley Periodicals LLC.)

Objective: Children with prolonged hospital admissions for CHD often develop delirium. Antipsychotic medications (APMs) have been used to treat delirium but are known to prolong the QTc duration. There is concern for prolongation of the QTc interval in cardiac patients who may be more vulnerable to electrocardiogram (ECG) changes and may have postoperative QTc prolongation already. The goal of this study was to determine the effect of APM on QTc duration in postoperative paediatric cardiac patients and determine the effect of quetiapine and risperidone in treating delirium and QTc prolongation., Design: Retrospective study, July 1, 2017-May 31, 2022., Setting: Tertiary children's hospital., Patients: Included were patients admitted to the paediatric cardiac ICU at Children's Healthcare of Atlanta., Interventions: None., Measurements and Main Results: ECGs, delirium scores, and drug information were collected. Delirium was defined as Cornell Assessment of Pediatric Delirium (CAPD) score >9. Mixed effect models were performed to evaluate the effect of surgery on QTc change and the effect of antipsychotics on QTc and CAPD changes. There were 139 children, 55% male and 67% surgical admissions. Median age was 5.9 months. Mean QTc increased after cardiac surgery by 18 ms (p = 0.014, 95% CI 3.65-32.4). There was no significant change in QTc after antipsychotic administration (p = 0.064). The mean CAPD score decreased (12.5-7.2; p < 0.001). Quetiapine had the most improvement in delirium, and risperidone had the least improvement (77.8%, n = 14; 37.8%, n = 34, respectively; p = 0.002)., Conclusions: The QTc interval did not have a statistically significant change after the administration of antipsychotics, while there was improvement in the CAPD score. APMs may be administered safely without significant prolongation of the QTc and are an effective treatment for delirium.

Background: For left-sided radiofrequency catheter ablation (LCA) in pediatrics, significant practice variability exists regarding anticoagulation and discharge practices. Given the lack of data in pediatric patients, the risks and benefits of these practices are not well defined., Objective: The purpose of this study was to evaluate the safety of same-day discharge and use of aspirin (ASA) in pediatric patients following LCA., Methods: We performed a retrospective cohort study of pediatric patients who underwent LCA from 2010 to 2020 at our institution. Discharge timing and ASA usage were based on operator preference. The primary outcome was incidence of postablation anticoagulation complications reported within 1 month of the procedure., Results: Three hundred seventy-six patients underwent LCA and met inclusion criteria. Median [25th, 75th percentiles] age was 13.9 [10.5, 16.2] years; 18 (4.7%) had a history of structural heart disease. The most common substrates for ablation were Wolff-Parkinson-White syndrome (183 patients [48.7%]), concealed accessory pathway (159 patients [42.3%]), and ectopic atrial tachycardia (10 patients [2.7%]). Three hundred thirty-eight patients (89.9%) were discharged on the day of LCA. Seventy-six patients (20.2%) were prescribed ASA at discharge. Of those who underwent follow-up (273 patients [72.6%]), 7 (2.7%) reported an anticoagulation complication (5 with hematoma, 2 with headache). One of these patients was prescribed ASA; none required readmission. There was no correlation between anticoagulation complications and same-day discharge or with ASA usage., Conclusion: Given the rare incidence of anticoagulation complications in pediatric patients undergoing LCAs, same-day discharge from the electrophysiology laboratory without anticoagulation should be considered., Competing Interests: Disclosures The authors have no conflicts of interest to disclose., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

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., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

The Fontan Udenafil Exercise Longitudinal (FUEL) trial showed that treatment with udenafil was associated with improved exercise performance at the ventilatory anaerobic threshold in children with Fontan physiology. However, it is not known how the initiation of phosphodiesterase 5 inhibitor therapy affects heart rate and blood pressure in this population. These data may help inform patient selection and monitoring after the initiation of udenafil therapy. The purpose of this study is to evaluate the effects of udenafil on vital signs in the cohort of patients enrolled in the FUEL trial. This international, multicenter, randomized, double-blind, placebo-controlled trial of udenafil included adolescents with single ventricle congenital heart disease who had undergone Fontan palliation. Changes in vital signs (heart rate [HR], systolic [SBP] and diastolic blood pressure [DBP]) were compared both to subject baseline and between the treatment and the placebo groups. Additional exploratory analyses were performed to evaluate changes in vital signs for prespecified subpopulations believed to be most sensitive to udenafil initiation. Baseline characteristics were similar between the treatment and placebo cohorts (n = 200 for each). The groups demonstrated a decrease in HR, SBP, and DBP 2 hours after drug/placebo administration, except SBP in the placebo group. There was an increase in SBP from baseline to after 6-min walk test in the treatment and placebo groups, and the treatment group showed an increase in HR (87.4 ± 15.0 to 93.1 ± 19.4 beats/min, p <0.01) after exercise. When comparing changes from baseline to the 26-week study visit, small decreases in both SBP (-1.9 ± 12.3 mm Hg, p = 0.03) and DBP (-3.0 ± 9.6 mm Hg, p <0.01) were seen in the treatment group. There were no clinically significant differences between treatment and placebo group in change in HR or blood pressure in the youngest age quartile, lightest weight quartile, or those on afterload-reducing agents. In conclusion, initiation of treatment with udenafil in patients with Fontan circulation was not associated with clinically significant changes in vital signs, implying that for patients similar to those enrolled in the FUEL trial, udenafil can be started without the requirement for additional monitoring after initial administration., Competing Interests: Declaration of Competing Interest The views expressed in this manuscript are those of the authors and do not necessarily represent the views of the National Heart, Lung, and Blood Institute; the National Institutes of Health; the U.S. Department of Health and Human Services; or Mezzion Pharma Co. Ltd., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: A subset of patients who develop post-surgical heart block have recovery of atrioventricular node function. Factors predicting recovery are not understood. We investigated our centre's incidence of post-surgical heart block and examine factors associated with recovery of atrioventricular node function., Methods: We conducted a single-centre retrospective study of patients 0 - 21 years who underwent cardiac surgery between January 2010 and December 2019 and experienced post-operative heart block. Data including patient and clinical characteristics and operative variables were collected and analysed., Results: Of 6333 surgical hospitalisations, 128 (2%) patients developed post-operative heart block. Of the 128 patients, 90 (70%) had return of atrioventricular node function, and 38 (30%) had pacemaker placement. Of the 38 patients who underwent pacemaker placement, 6 (15.8%) had recovery of atrioventricular node function noted on long-term follow-up. Median time from onset of heart block to late atrioventricular node recovery was 13 days (Interquartile range: 5 - 117). Patients with single-ventricle physiology (p = 0.04), greater weight (p = 0.03), and shorter cardiopulmonary bypass time (p = 0.015) were more likely to have recovery. The use of post-operative steroids was similar between all groups (p = 0.445). Infectious or wound complications were similar between pacemaker groups (p = 1)., Conclusions: Two per cent of patients who underwent congenital cardiac surgery developed post-operative heart block, and 0.6% underwent pacemaker placement. Early recovery of atrioventricular node was associated with greater weight at the time of surgery, single-ventricle physiology, and shorter cardiopulmonary bypass time. Late recovery of atrioventricular node conduction following pacemaker placement occurred in 15.8% of patients.

Background: Genetic defects in the RAS/mitogen-activated protein kinase pathway are an important cause of hypertrophic cardiomyopathy (RAS-HCM). Unlike primary HCM (P-HCM), the risk of sudden cardiac death (SCD) and long-term survival in RAS-HCM are poorly understood., Objectives: The study's objective was to compare transplant-free survival, incidence of SCD, and implantable cardioverter-defibrillator (ICD) use between RAS-HCM and P-HCM patients., Methods: In an international, 21-center cohort study, we analyzed phenotype-positive pediatric RAS-HCM (n = 188) and P-HCM (n = 567) patients. The between-group differences in cumulative incidence of all outcomes from first evaluation were compared using Gray's tests, and age-related hazard of all-cause mortality was determined., Results: RAS-HCM patients had a lower median age at diagnosis compared to P-HCM (0.9 years [IQR: 0.2-5.0 years] vs 9.8 years [IQR: 2.0-13.9 years], respectively) (P < 0.001). The 10-year cumulative incidence of SCD from first evaluation was not different between RAS-HCM and P-HCM (4.7% vs 4.2%, respectively; P = 0.59). The 10-year cumulative incidence of nonarrhythmic deaths or transplant was higher in RAS-HCM compared with P-HCM (11.0% vs 5.4%, respectively; P = 0.011). The 10-year cumulative incidence of ICD insertions, however, was 5-fold lower in RAS-HCM compared with P-HCM (6.9% vs 36.6%; P < 0.001). Nonarrhythmic deaths occurred primarily in infancy and SCD primarily in adolescence., Conclusions: RAS-HCM was associated with a higher incidence of nonarrhythmic death or transplant but similar incidence of SCD as P-HCM. However, ICDs were used less frequently in RAS-HCM compared to P-HCM. In addition to monitoring for heart failure and timely consideration of advanced heart failure therapies, better risk stratification is needed to guide ICD practices in RAS-HCM., Competing Interests: Funding Support and Author Disclosures The project was supported through funding from the Ted Rogers Centre for Heart Research, the Heart and Stroke Foundation/Robert M Freedom Chair, and the Canadian Institutes of Health Research to Dr Mital. Dr Lynch is supported by a Heart Failure Research Fellowship from Bristol Myers Squibb, Mitacs, and Myant. Dr Mital has served as a consultant for Bristol Myers Squibb and Tenaya Therapeutics; and has received unrestricted education funding from Bristol Myers Squibb. Dr Conway has served as a medical monitor for the PumpKIN trial; and has received unrestricted education funding from Abbott. Dr Rossano has served as a consultant for Merck, Bayer, Myokardia, and Cytokinetics. Dr Kantor has served as a consultant for Novartis and AstraZeneca. Dr Balaji has served as a consultant for Milestone Pharmaceuticals and Janssen Pharmaceuticals; and has received a research support grant from the Medtronic External Research Program. Dr Godown has served as a consultant for Daiichi-Sankyo. Dr Aziz has served on the Medical Advisory Board for Medtronic. Dr Jeewa has served as a medical monitor for the PumpKIN trial; and has served as a consultant for Abbott. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Hypertrophic cardiomyopathy (HCM) can be associated with an abnormal exercise response. In adults with HCM, abnormal results on exercise stress testing are predictive of heart failure outcomes. Our goal was to determine whether an abnormal exercise response is associated with adverse outcomes in pediatric patients with HCM., Methods: In an international cohort study including 20 centers, phenotype-positive patients with primary HCM who were <18 years of age at diagnosis were included. Abnormal exercise response was defined as a blunted blood pressure response and new or worsened ST- or T-wave segment changes or complex ventricular ectopy. Sudden cardiac death (SCD) events were defined as a composite of SCD and aborted sudden cardiac arrest. Using Kaplan-Meier survival, competing outcomes, and Cox regression analyses, we analyzed the association of abnormal exercise test results with transplant and SCD event-free survival., Results: Of 724 eligible patients, 630 underwent at least 1 exercise test. There were no major differences in clinical characteristics between those with or without an exercise test. The median age at exercise testing was 13.8 years (interquartile range, 4.7 years); 78% were male and 39% were receiving beta-blockers. A total of 175 (28%) had abnormal test results. Patients with abnormal test results had more severe septal hypertrophy, higher left atrial diameter z scores, higher resting left ventricular outflow tract gradient, and higher frequency of myectomy compared with participants with normal test results ( P <0.05). Compared with normal test results, abnormal test results were independently associated with lower 5-year transplant-free survival (97% versus 88%, respectively; P =0.005). Patients with exercise-induced ischemia were most likely to experience all-cause death or transplant (hazard ratio, 4.86 [95% CI, 1.69-13.99]), followed by those with an abnormal blood pressure response (hazard ratio, 3.19 [95% CI, 1.32-7.71]). Exercise-induced ischemia was also independently associated with lower SCD event-free survival (hazard ratio, 3.32 [95% CI, 1.27-8.70]). Exercise-induced ectopy was not associated with survival., Conclusions: Exercise abnormalities are common in childhood HCM. An abnormal exercise test result was independently associated with lower transplant-free survival, especially in those with an ischemic or abnormal blood pressure response with exercise. Exercise-induced ischemia was also independently associated with SCD events. These findings argue for routine exercise testing in childhood HCM as part of ongoing risk assessment.

Purpose/objectives: The coronavirus disease 2019 (COVID-19) pandemic has presented mental health challenges among healthcare professionals, including posttraumatic stress disorder (PTSD). Few studies have examined PTSD predictors in dental settings. This cross-sectional study aimed to describe the relationship between personality traits and PTSD symptoms among US dental residents engaged in patient care during the pandemic., Methods: An online survey was administered to residents in advanced education in general dentistry, dental anesthesia, general practice residency, oral and maxillofacial surgery, and pediatric dentistry programs between September 2020 and April 2021. The survey included a sociodemographic questionnaire, the Big Five Inventory, and the PTSD Checklist for Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition. Logistic regression models, analysis of variance, and Tukey tests were used to examine the association between variables and PTSD scores and assess differences in personality traits and PTSD by specialty., Results: Among 149 respondents (mean age = 29.9 ± 4.9 years; 57.0% female), 53.7% reported experiencing stress during the pandemic. Many residents (38.9%) were not aware of available mental health resources. A total of 17.4% of residents met the diagnostic criteria for PTSD, but only 1.3% reported a previous PTSD diagnosis. The most significant predictors of PTSD were neuroticism (odds ratio = 2.90, p = 0.046) and stress unrelated to the resident's program role during the pandemic (odds ratio = 5.88, p = 0.02)., Conclusions: PTSD symptoms were highly prevalent among dental residents, with stress and neuroticism being the most significant predictors., (© 2022 American Dental Education Association.)

Background: Cryoablation for atrioventricular nodal reentrant tachycardia (AVNRT) is effective and safe with a reported limitation of lower success and higher recurrence rates. We have observed cases in which slow pathway conduction was eliminated as demonstrated by atrial extra-stimulus testing within 1 min of cryo-energy delivery but returned following tissue rewarming. Frequently, slow pathway conduction persisted despite multiple acutely successful lesions over a broad anatomic region. We aimed to determine if return of slow pathway conduction after elimination during cryoablation represents a risk for recurrent AVNRT with the same intermediate term results as slow pathway ablation. We hypothesize that remnant single echo beats in the absence of sustained slow pathway conduction and inducible AVNRT is an acceptable end point after clear slow pathway elimination during cryoablation., Methods: Retrospective chart review of patients undergoing attempted slow pathway ablation for AVNRT using solely cryoablation between January 2015-January 2018., Results: Forty-four patients met inclusion criteria with at-least 2 features of dual AVN physiology. 19 patients had return of slow pathway conduction shortly after clear elimination during cryoablation (Group A) while 25 did not (Group B). All in Group A had recurrent single echo beats but none had sustained slow pathway conduction at the end of the procedure nor AVNRT recurrence at 1 year., Conclusion: Recurrent single echo beats with absent sustained slow pathway conduction and non-inducible AVNRT may be an acceptable endpoint for slow pathway ablation of AVNRT using cryoablation when there is elimination of slow pathway demonstrated during energy delivery., Competing Interests: Declaration of competing interest None of the authors have any conflicts of interest to disclose., (Copyright © 2020 Indian Heart Rhythm Society. Production and hosting by Elsevier B.V. All rights reserved.)

Introduction: Atrial flutter (AFL) and atrial fibrillation (AF) are common in pediatric patients with congenital heart disease and structurally normal hearts as well. Chemical cardioversion is attractive for patients with AFL/AF for a short period of time because of the ability to avoid sedation. We review a single center's experience with Ibutilide in pediatric patients in an effort to report on its safety and efficacy., Methods: We performed a retrospective chart review of pediatric patients (0-21 years) who underwent chemical cardioversion for AFL/AF with Ibutilide (January 2011-February 2019). Patients on another antiarrhythmic medication or attempted chemical cardioversion with another drug were excluded., Results: There were 21 patients who met inclusion criteria. Thirteen of the 21 (62%) patients were successfully cardioverted with Ibutilide (10 out of 13 had AF and four out of 13 required a second dose). There were no significant differences in baseline characteristics between those who were successfully cardioverted compared to those who were not. Administration of magnesium prior to administration did not appear to have an effect on the success rate. There was a significant increase in rate corrected QT interval (QTc) post Ibutilide administration, which returned to baseline prior to discharge. One patient had symptomatic bradycardia needing intravenous fluids and another had torsades requiring electrical cardioversion during Ibutilide administration., Conclusions: The success rate of chemical cardioversion with Ibutilide was similar in our experience as compared to studies in the adult population and the other lone pediatric study. Although adverse events were uncommon, Ibutilide administration warrants close monitoring and fully defining its efficacy warrants further pediatric experience., (© 2020 Wiley Periodicals, Inc.)

Introduction: Right ventricular pacing is associated with pacemaker induced cardiomyopathy and lesser degrees of pacing-induced LV dysfunction (PIVD) manifested by a reduction in left ventricular ejection fraction (LVEF). Our objective was to determine whether apical 4 chamber strain (A4C) by echocardiography can identify patients at risk of PIVD before LVEF declines., Methods and Results: A retrospective chart review of patients (0-21 years) who had a pacemaker with a ventricular lead placed between 2011 and 2017 was performed. Patients were divided into group A (LVEF <55% and/or >10% decline in LVEF within 12 months of pacemaker placement) and group B. Data have collected before and 1 and 12 months postpacemaker implantation. There were 30 patients in the group A and 60 in group B. At 1 and 12 months postpacemaker implantation, the LVEF was significantly lower while the A4C and QRS duration on electrocardiogram were significantly higher in the group A. While the LVEF and A4C became markedly abnormal in group A as early as 1 month, the A4C did not seem to demonstrate such marked abnormalities in group B. However, a sub-analysis of patients in the group A with preserved LVEF at 1 month demonstrated significant worsening in their A4C at that time., Conclusion: Myocardial deformation imaging may be a clinically useful tool for the prediction of a decline in LV systolic function following pacemaker implantation. Abnormalities in A4C seem to appear before LVEF decline and as soon as 1-month postpacemaker implantation., (© 2019 Wiley Periodicals, Inc.)

Background: Children with cardiac disease are at high risk for stroke. Approximately one-quarter of strokes in children with cardiac disease occur in the peri-procedural period; yet, the risk factors, clinical presentation, and treatment of post-catheterization stroke in children have not been well defined., Methods: We conducted a retrospective review of the medical records of patients aged zero to 18 years with a new clinically-apparent arterial ischemic stroke after cardiac catheterization at a tertiary children's hospital from 2006 to 2016. We excluded patients who had cardiac surgery, a cardiac arrest, extracorporeal membrane oxygenation, a ventricular assist device, or an arrhythmia proximate to their stroke., Results: Twenty children had a new clinically-apparent post-catheterization arterial ischemic stroke. The median age was one year (range, two days to 16 years). The most common procedures were balloon dilation for pulmonary vein stenosis (n = 6) and systemic pulmonary collateral closure (n = 5). The most common presenting symptoms were arm weakness (n = 10) and seizure (n = 8). The median time from catheterization to symptom discovery was 31.5 hours (interquartile range, 16.2 to 47.8 hours; n = 18). The median Pediatric Stroke Outcome Measure score 12 months post-stroke was 0.75 (range, 0 to 2; n = 6)., Conclusions: Although arterial ischemic stroke after cardiac catheterization is rare, better understanding this entity is important as children with cardiac disease and stroke have ongoing morbidity. Ameliorating this morbidity requires efforts aimed at preventing and rapidly detecting stroke, thereby enabling timely institution of neuroprotective measures and treatment with hyperacute therapies., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Transvenous pacemaker implantation for sinus node dysfunction in patients with Fontan palliation presents the difficulty of finding suitable pacing tissue and the potential of causing vascular obstruction in a low-flow circuit. We describe a patient who underwent electro-anatomic voltage mapping to guide a transvenous single chamber lead within her Fontan baffle. This highlights the use of advanced mapping technologies for pacemaker implantation in complex cyanotic heart disease.