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Background: An implantable cardioverter-defibrillator (ICD) in the pediatric patient (and the precipitating events that led to ICD placement) can be traumatic for patients and their families and may lead to posttraumatic stress disorder (PTSD)., Objectives: This study aimed to estimate the prevalence of PTSD in pediatric patients with an ICD and their parents and identify the factors associated with PTSD incidence., Methods: Pediatric participants with an ICD aged 8-21 years and parents of children aged 0-21 years completed surveys that included demographic characteristics and PTSD measures. Pediatric participants completed additional psychosocial measures, such as anxiety and depression self-report questionnaires., Results: Fifty youth (30% female) and 43 parents (70% female) completed the measures. Six of 50 youth (12%) met the screening criteria for a likely PTSD diagnosis, while 20 of 43 parents (47%) met the cutoff for PTSD on the screening measure. Children with PTSD were more likely to have had a secondary prevention ICD (83% vs 17%; P = .021), meet the clinical cutoff for depression (67% vs 16%; P = .005), and had higher shock anxiety scores (31.7 vs 17.9; P = .003) than children without PTSD. Female gender (57% vs 23%; P = .043) and patient depression (31% vs 5%; P = .042) were associated with PTSD in parents., Conclusion: Parents were found to be more likely to meet the criteria for PTSD than youth. In youth, PTSD was associated with medical and psychosocial factors, whereas PTSD in parents was associated with being female and child depression. Clinic-based screenings and management planning of emotional functioning are warranted to address psychological distress in patients and parents., (Copyright © 2022 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Abrupt loss of ventricular preexcitation on noninvasive evaluation, or nonpersistent preexcitation, in Wolff-Parkinson-White syndrome (WPW) is thought to indicate a low risk of life-threatening events., Objective: The purpose of this study was to compare accessory pathway (AP) characteristics and occurrences of sudden cardiac arrest (SCA) and rapidly conducted preexcited atrial fibrillation (RC-AF) in patients with nonpersistent and persistent preexcitation., Methods: Patients 21 years or younger with WPW and invasive electrophysiology study (EPS) data, SCA, or RC-AF were identified from multicenter databases. Nonpersistent preexcitation was defined as absence/sudden loss of preexcitation on electrocardiogram, Holter monitoring, or exercise stress test. RC-AF was defined as clinical preexcited atrial fibrillation with shortest preexcited R-R interval (SPERRI) ≤ 250 ms. AP effective refractory period (APERP), SPERRI at EPS , and shortest preexcited paced cycle length (SPPCL) were collected. High-risk APs were defined as APERP, SPERRI, or SPPCL ≤ 250 ms., Results: Of 1589 patients, 244 (15%) had nonpersistent preexcitation and 1345 (85%) had persistent preexcitation. There were no differences in sex (58% vs 60% male; P=.49) or age (13.3±3.6 years vs 13.1±3.9 years; P=.43) between groups. Although APERP (344±76 ms vs 312±61 ms; P<.001) and SPPCL (394±123 ms vs 317±82 ms; P<.001) were longer in nonpersistent vs persistent preexcitation, there was no difference in SPERRI at EPS (331±71 ms vs 316±73 ms; P=.15). Nonpersistent preexcitation was associated with fewer high-risk APs (13% vs 23%; P<.001) than persistent preexcitation. Of 61 patients with SCA or RC-AF, 6 (10%) had nonpersistent preexcitation (3 SCA, 3 RC-AF)., Conclusion: Nonpersistent preexcitation was associated with fewer high-risk APs, though it did not exclude the risk of SCA or RC-AF in children with WPW., (Copyright © 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Pectoral nerve blocks (PECs) can reduce intraprocedural anesthetic requirements and postoperative pain. Little is known about the utility of PECs in reducing pain and narcotic use after pacemaker (PM) or implantable cardioverter-defibrillator (ICD) placement in children., Objective: The purpose of this study was to determine whether PECs can decrease postoperative pain and opioid use after PM or ICD placement in children., Methods: A single-center retrospective review of pediatric patients undergoing transvenous PM or ICD placement between 2015 and 2020 was performed. Patients with recent cardiothoracic surgery or neurologic/developmental deficits were excluded. Demographics, procedural variables, postoperative pain, and postoperative opioid usage were compared between patients who had undergone PECs and those who had undergone conventional local anesthetic (Control)., Results: A total of 74 patients underwent PM or ICD placement; 20 patients (27%) underwent PECs. There were no differences between PECs and Control with regard to age, weight, gender, type of device placed, presence of congenital heart disease, type of anesthesia, procedural time, or complication rates. Patients who underwent PECs had lower pain scores at 1, 2, 6, 18, and 24 hours compared to Control. PECs patients had a lower mean cumulative pain score [PECs 1.5 (95% confidence interval [CI] 0.8-2.2) vs Control 3.1 (95% CI 2.7-3.5); P <.001] and lower total opioid use [PECs 6.0 morphine milligram equivalent (MME)/m 2 (95% CI 3.4-8.6) vs Control 15.0 MME/m 2 (95% CI 11.8-18.2); P = .001] over the 24 hours postimplant., Conclusion: PECs reduce postoperative pain scores and lower total opioid usage after ICD or PM placement. PECs should be considered at the time of transvenous device placement in children., (Copyright © 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: The postpacing interval (PPI) minus the tachycardia cycle length (TCL) is frequently used to investigate tachycardias. However, a variety of issues (eg, failure to entrain, decremental conduction, and oscillating TCLs) can make interpretation of the PPI-TCL challenging., Objective: The purpose of this study was to investigate a novel maneuver to confirm the PPI-TCL value without using either the ventricular PPI or the TCL interval and to assess the ability of this maneuver to identify decremental conduction and differentiate supraventricular tachycardias., Methods: We analyzed 77 intracardiac recordings from patients (age 25 ± 20 years; 40 female) who underwent catheter ablation of atrioventricular nodal reentrant tachycardia (AVNRT) or orthodromic reciprocating tachycardia (ORT) with a concealed pathway. We calculated the PPI-TCL, the AH-corrected PPI-TCL, and estimated the PPI-TCL using "dual-chamber entrainment" calculated as [PPI V - TCL = Stim(A→V) + Stim(V→A) - PPI A ]., Results: The PPI-TCL calculated by dual-chamber entrainment highly correlated with the observed and AH-corrected PPI-TCL (R 2  = 0.79 and 0.96, respectively; P <.001]. A dual-chamber entrainment PPI-TCL value of 80 ms correctly differentiated all AVNRT from septal ORT cases, whereas the standard PPI-TCL and AH-corrected PPI-TCL methods were incorrect in 14% and 6% of cases, respectively. Dual-chamber entrainment identified 3 ± 10 ms of additional decremental conduction beyond AH prolongation, including 4 pathways with significant (>10 ms) decrement., Conclusion: Dual-chamber entrainment estimates the PPI-TCL value without using either the ventricular PPI or the TCL interval. This maneuver adjusts for all decremental conduction, including within concealed pathways, where a dual-chamber entrainment PPI-TCL value >80 ms favors AVNRT over ORT. This maneuver can be used to verify the observed PPI-TCL value in challenging cases., (Copyright © 2018 Heart Rhythm Society. All rights reserved.)

Background: Cryoablation for atrioventricular nodal reentrant tachycardia (AVNRT) is associated with higher recurrence rates than radiofrequency ablation (RFA). Junctional tachycardia marks procedural success with RFA, but no such indicator exists for cryoablation., Objective: The purpose of this study as to determine the impact of voltage mapping plus longer ablation lesions on midterm success of cryoablation for children with AVNRT., Methods: We performed a single-center retrospective analysis of pediatric patients with AVNRT who underwent cryoablation from 2011 to 2015. Patients ablated using a standard electroanatomic approach (control) were compared with patients ablated using voltage mapping (voltage group). In the voltage group, EnSite NavX navigation and visualization technology (St Jude Medical, St Paul, MN) was used to develop a "bridge" of lower voltage gradients (0.3-0.8 mV) of the posteroseptal right atrium to guide cryoablation. Kaplan-Meier analysis was used to determine freedom from recurrence of supraventricular tachycardia., Results: In all, 122 patients were included (71 voltage, 51 control). There was no difference between groups regarding age, sex, or catheter-tip size. Short-term success was similar in both groups (98.5% voltage vs 92% control; P = .159), but recurrence rates were lower in the voltage group (0% vs 11%, P = .006). Follow-up time was shorter in the voltage group (15 ± 7 months vs 22 ± 17 months, P < .05). The 1-year freedom from recurrence was lower in the voltage group (100% vs 91.5%, P <.05). Ablation times were longer in the voltage group (43.7 ± 20.9 minutes vs 34.3 ± 20.5 minutes, P = .01), but overall procedure times were shorter in the voltage group (157 ± 40 minutes vs 198 ± 133 minutes; P = .018). No significant complication was seen in either group., Conclusion: Voltage gradient mapping and longer lesion time can decrease recurrence rates in pediatric patients with AVNRT., (Copyright © 2017 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Chronic ventricular pacing (VP) is associated with systolic dysfunction in a subset of pediatric patients with heart block and structurally normal hearts. The effect of chronic VP in congenital heart disease is less well understood, specifically in the single-ventricle (SV) population., Objective: To determine the longitudinal effect of VP in SV patients., Methods: SV patients with heart block and dual-chamber pacemakers requiring >50% VP were compared with nonpaced (controls) SV patients matched for age, sex, and SV morphology. Patients were excluded if a prepacing echocardiogram was not available. Echocardiogram and clinical parameters were compared at baseline (prepacing) and at last follow-up in the paced group, and in controls when they were at ages similar to those of their paced-group matches., Results: Twenty-two paced and 53 control patients from 2 institutions were followed for similar durations (6.6±5 years vs 7.6±7.6 years; P = .59). There was no difference between groups regarding baseline ventricular function or the presence of moderate-to-severe atrioventricular valvar regurgitation (AVVR). Paced patients were more likely to develop moderate-to-severe systolic dysfunction (68% vs 15%; P < .01) and AVVR (55% vs 8%; P < .001) and require heart failure medications (65% vs 21%; P < .001). Chronic VP was also associated with a higher risk of transplantation or death (odds ratio, 4.9; 95% confidence interval, 1.05-22.7; P = .04)., Conclusions: SV patients requiring chronic VP are at higher risk of developing moderate-to-severe ventricular dysfunction and AVVR with an increased risk of death or transplantation compared with controls. New strategies to either limit VP or improve synchronization in this vulnerable population is imperative., (Copyright © 2017 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Life-threatening arrhythmia events (LTEs) occur in ~5% of children with dilated cardiomyopathy (DCM). While prolonged QRS duration has been shown to be associated with LTEs, electrocardiographic (ECG) repolarization findings have not been examined., Objective: We sought to determine the associations between ECG repolarization abnormalities and LTEs in children with DCM., Methods: A single-center retrospective review of children with DCM was performed. LTEs were defined as documented ventricular tachycardia or fibrillation requiring medical intervention. Three pediatric cardiologists, blinded to clinical events, evaluated ECGs obtained at the time of initial referral. Kaplan-Meier survival and Cox proportional hazards analyses were used to evaluate time to LTEs., Results: A total of 137 patients (mean age 7.8 ± 6.7 years; 75(55%) male patients) with DCM (mean ejection fraction 35% ± 16%) were included; 67 patients (49%) had a corrected JT (JTc) interval of ≥340 ms, 72 (53%) had a corrected QT (QTc) interval of ≥450 ms, and 41 (30%) had abnormal T waves. LTEs occurred in 15 patients at a median of 12 months (interquartile range 3-36 months) after the initial ECG. Patients with LTEs had a longer JTc interval (371 ± 77 ms vs 342 ± 41 ms; P = .02) and a longer QTc interval (488 ± 96 ms vs 453 ± 44 ms; P = .01). In survival analysis, a JTc interval of ≥390 ms (hazard ratio [HR] 4.07; 95% confidence interval [CI] 1.12-14.83; P = .03), a QTc interval of ≥510 ms (HR 6.95; 95% CI 1.53-31.49; P = .01), abnormal T-wave inversion (HR 11.62; 95% CI 2.75-49.00; P = .001), and ST-segment depression (HR 6.91; 95% CI 1.25-38.27; P = .03) were associated with an increased risk of LTEs, even after adjusting for QRS duration and amiodarone use., Conclusion: Repolarization abnormalities are common in children with DCM. Certain ECG repolarization abnormalities, such as significantly prolonged JTc and QTc intervals, may be useful in identifying patients at risk of LTEs., (Copyright © 2016 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: One of the basic electrophysiological principles of atrioventricular reciprocating tachycardia (AVRT) is that ventriculoatrial (VA) times during tachycardia are >70 ms. We hypothesized, however, that children may commonly have VA times <70 ms in AVRT., Objective: This study sought to determine the incidence and characteristics associated with short-VA AVRT in children., Methods: A retrospective single-center review of children with AVRT from 2000 to 2014 was performed. All patients ≤18 years of age with AVRT at electrophysiology study were included. Patients with persistent junctional reciprocating tachycardia, atrioventricular nodal reentry tachycardia, and tachycardia not unequivocally proven to be AVRT were excluded. VA time was defined as the time between earliest ventricular activation and earliest atrial activation in any lead and was confirmed by 2 electrophysiologists. Patients with VA times <70 ms (SHORT-VA) and those with standard VA times ≥70 ms (STD-VA) were compared. Logistic regression analysis identified characteristics of SHORT-VA patients., Results: A total of 495 patients with AVRT were included (mean age 11.7 ± 4.1 years). There were 265 patients (54%) with concealed accessory pathways (APs) and 230 (46%) with Wolff-Parkinson-White syndrome. AP location was left-sided in 301 patients (61%) and right-sided in 194 (39%). The mean VA time in AVRT was 100 ± 33 ms. A total of 63 patients (13%) had VA times <70 ms (SHORT-VA). The shortest VA time during AVRT was 50 ms. There was no difference in age, AV nodal block cycle, or body surface area between SHORT-VA and STD-VA patients, but SHORT-VA patients had lower weight (43 ± 17 vs 51 ± 23 kg, P = .02), lower AV nodal effective refractory period (AVNERP; 269 ± 50 vs 245 ± 52 ms, P < .01), and more left-sided APs (50 [79%] vs 251 [58%]; P < .01]. On multivariate logistic regression, factors associated with SHORT-VA included left-sided AP (odds ratio [OR] 5.79, confidence interval [95% CI] 2.21-15.1, P < .01), shorter AVNERP (OR 0.99, CI 0.98-0.99, P < .01), and lower weight (OR 0.97, CI 0.95-0.99, P < .01)., Conclusions: Children with AVRT can frequently have VA times <70 ms, with 50 ms being the shortest VA time. This finding debunks the classic electrophysiology principle that VA times in AVRT must be >70 ms. SHORT-VA AVRT was more common in children with left-sided APs., (Copyright © 2015 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Chronic right ventricular (RV) pacing has been associated with decreased exercise capacity and left ventricular (LV) function in adults with congenital complete atrioventricular block (CCAVB), but not in children., Objective: The purpose of this study was to evaluate the exercise capacity and LV function in pediatric patients with CCAVB receiving chronic RV pacing., Methods: We prospectively evaluated pediatric patients with isolated CCAVB receiving atrial synchronous RV pacing for at least 5 years. Supine bicycle ergometry was performed, and LV ejection fraction (EF) was evaluated by echocardiography., Results: Ten CCAVB subjects and 31 controls were matched for age, gender, and body surface area. CCAVB subjects had normal resting EF (63.1% ± 4.0%) and had been paced for 7.9 ± 1.4 years. Exercise testing demonstrated reduced functional capacity in CCAVB patients compared to controls with a lower VO₂peak (26.0 ± 6.6 mL/kg/min vs 39.9 ± 7.0 mL/kg/min, P <.001), anaerobic threshold (15.6 ± 3.9 mL/kg/min vs 18.8 ± 2.7 mL/kg/min, P = .007), and oxygen uptake efficiency slope (1210 ± 406 vs 1841 ± 452, P <.001). Maximum heart rate (165 ± 8 bpm vs 185 ± 9 bpm, P <.001) and systolic blood pressure (159 ± 17 mm Hg vs 185 ± 12 mm Hg, P <.019) also were reduced in CCAVB patients despite maximal effort (respiratory exchange ratio 1.2 ± 0.1). EF was augmented with exercise in controls but not in CCAVB patients (13.2% ± 9.3% vs 0.2% ± 4.8% increase, P <.001)., Conclusion: Clinically asymptomatic children with chronic RV pacing due to CCAVB have significant reductions in functional capacity accompanied by chronotropic incompetence and inability to augment EF with exercise., (Copyright © 2015 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Children requiring a permanent epicardial pacemaker (PM) traditionally have a single lead placed on the right ventricle. Lead failure in pacemaker-dependent (PMD) children, however, can result in cardiovascular events (CVEs) and death., Objective: The purpose of this study was to determine if redundant ventricular lead systems (RVLS) can safeguard against CVE and death in PMD children., Methods: This was a single-center study of PMD patients undergoing placement of RVLS from 2002-2013. Patients ≤21 years of age who were PMD were included. Patients with a biventricular (BiV) system placed for standard resynchronization indications were excluded. RVLS patients were compared to PMD patients with only a single pacing lead on the ventricle (SiV)., Results: Seven hundred sixty-nine patients underwent PM/implantable cardioverter-defibrillator placement with 76 BiV implants; 49 patients (6%) were PMD. Thirteen patients underwent implantation of an RVLS. There was no difference between the RVLS group (n = 13) and SiV PMD control group (n = 24) with regard to age (RVLS 9.5 ± 5.8 years vs SiV 9.4 ± 6.7 years, P = .52), weight (RVLS 38.2 ± 32.6 kg vs SiV 35.2 ± 29.3 kg, P = .62), indication for pacing, procedural complications, or time to follow-up. There were 2 lead fractures (17%) in the RVLS group (mean follow-up 3.8 ± 2.9 years), with no deaths or presentations with CVE. The SiV control group had 3 lead fractures (13%) (mean follow-up 2.8 ± 2.9 years), with no deaths, but all 3 patients presented with CVE and required emergent PM placement., Conclusion: RVLS systems should be considered in children who are PMD and require permanent epicardial pacing. BiV pacing and RVLS may decrease the risk of CVE in the event of lead failure in PMD patients., (Copyright © 2015 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Cardiac resynchronization therapy has been proposed for treatment of hypoplastic left heart syndrome (HLHS) patients with right ventricular (RV) failure. The role of dyssynchrony, however, is poorly understood in this population., Objective: The purpose of this study was to better understand the relationship between electrical and mechanical dyssynchrony in HLHS using 3-dimensional electrical mapping, tissue Doppler indices of wall motion, and vector velocity imaging., Methods: Eleven HLHS subjects with normal RV function and ten normal subjects (age 3-18 years) were studied. Electrical and mechanical activation times and dyssynchrony indices (electrical dyssynchrony index, mechanical dyssynchrony index) were calculated using 3-dimensional electrical mapping, tissue Doppler indices, and vector velocity imaging., Results: No differences in measures of electrical dyssynchrony were seen when comparing HLHS patients and normal patients (electrical activation time 63.3 ± 22.8 ms vs 56.2 ± 11.2 ms, P = .38; electrical dyssynchrony index 13.7 ± 6.3 ms vs 11.6 ± 3.0 ms, P = .34). However, measures of mechanical dyssynchrony were markedly abnormal in HLHS patients despite normal RV function (mechanical activation time 16 ± 11.3 ms vs 0.9±1.9 ms, P = .01; mechanical dyssynchrony index 7.5 ± 5.5 vs 0.4 ± 0.8, P<.01)., Conclusion: Patients with HLHS and preserved RV systolic function have normal electrical activation when compared to patients with normal right and left ventricles. In contrast, these patients demonstrate mechanical dyssynchrony compared to patients with normal right and left ventricles. This finding raises important questions about the indications for cardiac resynchronization therapy in this patient population., (Copyright © 2012 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)