Incidence and outcome of arrhythmias and electrical disease in patients with Trisomy 18.

Patients with Trisomy 18 have a high incidence of cardiac anomalies and are associated with early death. Because of early mortality, electrical system disease and arrhythmia has been difficult to delineate and the incidence remain unknown. We sought to describe the association and clinical outcomes of electrical system disease and cardiac tachy-arrhythmias in patients with Trisomy 18. This was a retrospective, single institutional study. All patients with Trisomy 18 were included in the study. Patient characteristics, congenital heart disease (CHD), conduction system and clinical tachy-arrhythmia data were collected on all patients. Outcomes including cardiac surgical interventions, electrical system interventions and death were collected until the time of study. Patients with tachy-arrhythmias/electrical system involvement were compared to those without to identify potential associated variables. A total of 54 patients with Trisomy 18 were included in analysis. The majority of patients was female and had associated CHD. AV nodal conduction system abnormalities with either first or second degree AV block were common (15%) as was QTc prolongation (37%). Tachy-arrhythmias were common with 22% of patients having at least one form of tachy-arrhythmia and associated with concomitant conduction system disease (p = 0.002). Tachy-arrhythmias were typically treatable with monitoring or medication with eventual resolution without need for procedural intervention. Although early death was common, there were no causes of death associated with tachy-arrhythmia or conduction system disease. In conclusion, patients with Trisomy 18 have a high incidence of conduction system abnormalities and burden of clinical tachy-arrhythmias. Although frequent, electrical system disease did not affect patient outcome or difficultly of care delivery.

Transesophageal pacing studies reduce readmission but prolong initial admission in infants with supraventricular tachycardia: A cost-comparison analysis.

Background: Supraventricular tachycardia (SVT) is a common arrhythmia. Infants with SVT are often admitted to initiate antiarrhythmics. Transesophageal pacing (TEP) studies can be used to guide therapy prior to discharge. Objective: The objective of this study was to investigate the impact of TEP studies on length of stay (LOS), readmission, and cost in infants with SVT. Methods: This was a 2-site retrospective review of infants with SVT. One site (Center TEPS) utilized TEP studies in all patients. The other (Center NOTEP) did not. Patients with structural heart disease, patients with gestational age <34 weeks, and patients diagnosed after 6 months were excluded. At Center TEPS, repeat TEP studies were performed after titration of medication until SVT was not inducible. Primary endpoints were LOS and readmission for breakthrough SVT within 31 days of discharge. Hospital reimbursement data were utilized for cost-effectiveness analysis. Results: The cohort included 131 patients, 59 in Center TEPS and 72 in Center NOTEP. One patient was readmitted in Center TEPS vs 17 in Center NOTEP (1.6% vs 23.6%; P ≤ .001). Median LOS was longer for Center TEPS at 118.0 (interquartile range [IQR] 74.0-189.5) hours vs Center NOTEP at 66.9 (IQR 45.5-118.3) hours (P = .001). Twenty-one patients had multiple TEP studies. Median length of readmission for Center NOTEP was 65 (IQR 41-101) hours. Including readmission costs, utilization of TEP studies resulted in a probability-weighted cost of $45,531 per patient compared with $31,087 per patient without TEP studies. Conclusion: Utilization of TEP studies was associated with decreased readmission rates but longer LOS and greater cost compared with SVT management without TEP studies.

Outcomes in Infants with Supraventricular Tachycardia: Risk Factors for Readmission, Recurrence and Ablation.

Supraventricular tachycardia (SVT) is the most common arrhythmia in infants. Once diagnosed, infants are admitted for antiarrhythmic therapy and discharged after observation. There are limited data on risk factors for readmission and readmission rates, while on medication. The objective of this study was to investigate risk factors for readmission and outcomes in infants diagnosed with SVT. This is a single-center retrospective study over a 10-year period of infants under 6 months of age with documented SVT. Infants with congenital heart disease requiring surgical or catheter intervention, gestational age less than 32 weeks or diagnosis of atrial flutter or fibrillation were excluded. The primary outcome was readmission within 31 days of hospital discharge. Long term need for ablation and eventual discontinuation of medications were assessed. Ninety patients were included. Beta blockers were the initial therapy in 66 and 28 required a medication change. Nineteen were readmitted within 31 days of discharge. The only clinical factor associated with early readmission was presence of ventricular pre-excitation (6/19 vs. 8/71, p = 0.03). Patients who were readmitted within 31 days had a longer length of treatment (12 [11.5, 22.0] vs. 10 [7.5, 12.0] months, p = 0.007) and were more likely to undergo ablation (4/19 vs. 2/71, p = 0.017). In this cohort of infants with SVT, readmission was common and ventricular pre-excitation was identified as a risk factor for readmission. Infants who were readmitted within 31 days of discharge had longer length of antiarrhythmic therapy and were more likely to undergo catheter ablation.

Predictors and Outcomes of Arrhythmia on Stage I Palliation of Single Ventricle Patients.

BACKGROUND: Arrhythmias are common in single ventricle patients though their effect on outcomes during stage I palliation (S1P) is unclear. OBJECTIVES: The authors sought to study associated risks for arrhythmia in patients undergoing S1P for single ventricle disease and evaluate the outcome of arrhythmias and their treatment strategies on survival. METHODS: Retrospective patient, surgical, medication, and arrhythmia data were obtained from the NPC-QIC (National Pediatric Cardiology Quality Improvement Collaborative) database. Bivariate analysis of variables associated with arrhythmias, as well as those associated with survival, was performed at the time of stage II palliation. Appropriate variables were included in multivariate modeling. RESULTS: Of the 2,048 patients included in the study, 36% had arrhythmia noted during their S1P hospitalization, with supraventricular tachycardia (12%) and focal atrial tachycardia (11%) the most common. At S1P discharge, 11% of patients were on an antiarrhythmic medication. Arrhythmias were associated with lower survival and increased hospital length of stay. Heterotaxy syndrome, younger age at S1P, male sex, and additional anomalies were associated with increased risk of arrhythmia in multivariable modeling (P ≤ 0.01). Arrhythmia and female sex were associated with increased mortality, whereas antiarrhythmic medication and digoxin use were associated with decreased mortality (P ≤ 0.003, model area under the curve = 0.79). The use of antiarrhythmic medications within the subcohort of arrhythmia patients was also associated with decreased risk of mortality (P < 0.0001; odds ratio: 2.0-7.2). CONCLUSIONS: Arrhythmias are common during admission for S1P and associated with poor outcomes. The use of antiarrhythmic medications may improve survival, though future studies are needed.

Continuous Arrhythmia Monitoring in Pediatric and Adult Patients With Left Ventricular Noncompaction.

Patients with left ventricular noncompaction (LVNC) are at risk of clinically significant arrhythmias and sudden death. We evaluated whether implantable loop recorders could detect significant arrhythmias that might be missed in these patients during annual Holter monitoring. Selected pediatric and adult patients with LVNC who consented to implantable loop recorder placement were monitored for 3 years (study duration, 10 April 2014-9 December 2019). Fourteen subjects were included (age range, 6.5-36.4 yr; 8 males). Of 13 patients who remained after one device extrusion, one underwent implantable cardioverter-defibrillator placement. Four patients (31%) had significant arrhythmias: atrial tachycardia (n=2), nonsustained ventricular tachycardia (n=1), and atrial fibrillation (n=1). All 4 events were clinically asymptomatic and not associated with left ventricular ejection fraction. In addition, a high frequency of benign arrhythmic patterns was detected. Implantable loop recorders enable continuous, long-term detection of important subclinical arrhythmias in selected patients who have LVNC. These devices may prove to be most valuable in patients who have LVNC and moderate or greater ventricular dysfunction.

Multicenter Outcomes of Catheter Ablation for Atrioventricular Reciprocating Tachycardia Mediated by Twin Atrioventricular Nodes.

OBJECTIVES: This study sought to describe the electrophysiologic properties and catheter ablation outcomes for atrioventricular reciprocating tacchycardia via twin atrioventricular nodes (T-AVRT). BACKGROUND: Although catheter ablation for T-AVRT is an established entity, there are few data on the electrophysiological properties and outcomes of this procedure. METHODS: An international, multicenter study was conducted to collect retrospective procedural and outcomes data for catheter ablation of T-AVRT. RESULTS: Fifty-nine patients with T-AVRT were identified (median age at procedure, 8 years [interquartile range: 4.4-17.0 years]; 49% male). Of these, 55 (93%) were diagnosed with heterotaxy syndrome (right atrial isomerism in 39, left atrial isomerism in 8, and indeterminate in 8). Twenty-three (39%) had undergone Fontan operation (12 extracardiac, 11 lateral tunnel). After the Fontan operation, atrial access was conduit or baffle puncture in 15 (65%), fenestration in 5 (22%), and retrograde in 3 (13%). Acute success was achieved in 43 (91%) of 47 attempts (targeting an anterior node in 23 and posterior node in 24). There was no high-grade AV block or change in QRS duration. Over a median of 3.8 years, there were 3 recurrences. Of 7 patients with failed index procedure or recurrent T-AVRT, 6 (86%) were associated with anatomical hurdles such as prior Fontan or catheter course through an interrupted inferior vena cava-to-azygous vein continuation (P = 0.11). CONCLUSIONS: T-AVRT can be targeted successfully with low risk for recurrence. Complications were rare in this population. Anatomical challenges were common among patients with reduced short and long-term efficacy, representing opportunities for improvement in procedural timing and planning.

Cumulative In-Hospital Costs Associated With Single-Ventricle Palliation.

Background: In the SVR (Single Ventricle Reconstruction) Trial, 1-year survival in recipients of right ventricle to pulmonary artery shunts (RVPAS) was superior to that in those receiving modified Blalock-Taussig-Thomas shunts (MBTTS), but not in subsequent follow-up. Cost analysis is an expedient means of evaluating value and morbidity. Objectives: The purpose of this study was to evaluate differences in cumulative hospital costs between RVPAS and MBTTS. Methods: Clinical data from SVR and costs from Pediatric Health Information Systems database were combined. Cumulative hospital costs and cost-per-day-alive were compared serially at 1, 3, and 5 years between RVPAS and MBTTS. Potential associations between patient-level factors and cost were explored with multivariable models. Results: In total, 303 participants (55% of the SVR cohort) from 9 of 15 sites were studied (48% MBTTS). Observed total costs at 1 year were lower for MBTTS ($701,260 ± 442,081) than those for RVPAS ($804,062 ± 615,068), a difference that was not statistically significant (P = 0.10). Total costs were also not significantly different at 3 and 5 years (P = 0.21 and 0.32). Similarly, cost-per-day-alive did not differ significantly for either group at 1, 3, and 5 years (all P > 0.05). In analyses of transplant-free survivors, total costs and cost-per-day-alive were higher for RVPAS at 1 year (P = 0.05 for both) but not at 3 and 5 years (P > 0.05 for all). In multivariable models, aortic atresia and prematurity were associated with increased cost-per-day-alive across follow-up (P < 0.05). Conclusions: Total costs do not differ significantly between MBTTS and RVPAS. The magnitude of longitudinal costs underscores the importance of efforts to improve outcomes in this vulnerable population.

Outcomes of Implantable Loop Monitoring in Patients <21 Years of Age.

Rhythm-symptom correlation in pediatric patients with syncope/palpitations or at risk cohorts can be difficult, but important given potential associations with treatable or malignant arrhythmia. We sought to evaluate the use, efficacy and outcomes of implantable loop recorders (ILR) in pediatrics. We conducted a retrospective study of pediatric patients (<21 years) with implanted ILR. Patient/historical characteristics and ILR indication were obtained. Outcomes including symptom documentation, arrhythmia detection and ILR based changes in medical care were identified. Comparison of outcomes were performed based on implant indication. Additional sub-analyses were performed in syncope-indication patients comparing those with and without changes in clinical management. A total of 116 patients with ILR implant were identified (79 syncope/37 other). Symptoms were documented 58% of patients (syncope 68% vs nonsyncope 35%; p = 0.002). A total of 37% of patients had a documented clinically significant arrhythmia and 25% of patients had a resultant change in clinical management independent of implant indication. Arrhythmia type was dependent on implant indication with nonsyncope patients having more ventricular arrhythmias. Pacemaker/defibrillator implantation and mediation management were the majority of the clinical changes. In conclusion, IRL utilization in selected pediatric populations is associated with high efficacy and supports clinical management. ILR efficacy is similar regardless of indication although patients with nonsyncope indications had a higher frequency of ventricular arrhythmias as opposed to asystole and heart block in syncope indications. The majority of arrhythmic findings occurred in the first 12 months, and new technology that would allow for less invasive monitoring for 6 to 12 months may be of value.

Predictors and outcomes of heart block during surgical stage I palliation of patients with a single ventricle: A report from the NPC-QIC.

BACKGROUND: Mortality in cohorts with a single ventricle remains high with multiple associated factors. The effect of heart block during stage I palliation remains unclear. OBJECTIVE: The purpose of this study was to study patient and surgical risks of heart block and its effect on 12-month transplant-free survival in patients with a single ventricle. METHODS: Patient, surgical, outcome data and heart block status (transient and permanent) were obtained from the National Pediatric Cardiology Quality Improvement Collaborative single ventricle database. Bivariate analysis was performed comparing patients with and without heart block, and multivariate modeling was used to identify variables associated with block. One-year outcomes were analyzed to identify variables associated with lower 12-month transplant-free survival. RESULTS: In total, 1423 patients were identified, of whom 28 (2%) developed heart block (second degree or complete) during their surgical admission. Associated risk factors for block included heterotaxy syndrome (odds ratio [OR] 6.4) and atrial flutter/fibrillation (OR 3.8). Patients with heart block had lower 12-month survival, though only in patients with complete heart block as opposed to second degree block. At 12 months of age, 43% (12/28) of patients with heart block died and were more likely to experience mortality at 12 months than patients without block (OR 4.9; 95% confidence interval 1.4-17.5; P = .01). CONCLUSION: Although rare, complete heart block after stage I palliation represents an additional risk of poor outcomes in this high-risk patient population. Heterotaxy syndrome was the most significant risk factor for the development of heart block after stage I palliation. The role of transient block in outcomes and potential rescue with long-term pacing remains unknown and requires additional study.

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.

Progressive QRS Duration and Ventricular Dysfunction in Pediatric Patients with Chronic Ventricular Pacing.

Pacemakers are a mainstay of therapy for patients with congenital and acquired heart block, but ventricular pacing is related to ventricular dysfunction. We sought to evaluate patient and device characteristics associated with ventricular dysfunction in pediatric patients with chronic ventricular pacing. This was a retrospective cohort of pediatric patients with heart block and chronic ventricular pacing. Patient, ECG, and device characteristics were analyzed to determine factors associated with ventricular dysfunction. Longitudinal ECG and echocardiogram parameters were obtained to track changes in QRS and systemic ventricular systolic function over time. In total, 82 patients were included (median age at implant 0.81 years). Over a follow-up time of 6.1 years, 18% developed ventricular dysfunction. Patients with dysfunction had greater current QRS duration (p = 0.002) compared to those with preserved function with a similar time from device implantation. There was no difference between lead location or age at device implantation. QRS duration increased with time from implant and the resultant ΔQRS was associated with ventricular dysfunction (p = 0.01). QRS duration >162 ms was associated with a 5.8 (2-9)-fold increased risk for dysfunction. Transvenous leads were associated with longer QRS duration with no difference compared to epicardial leads in development of ventricular dysfunction. This study demonstrated that the absolute paced QRS duration and Δpaced QRS were association with long-term ventricular dysfunction independent of how long a given patient was paced. Patients in high-risk categories may benefit from close echocardiographic monitoring. Whether permissive junctional rhythm or His bundle/biventricular pacing decreases the rate of dysfunction needs further study.

Wearable cardioverter-defibrillators in pediatric cardiomyopathy: A cost-utility analysis.

BACKGROUND: Dilated cardiomyopathy (DCM) is the most common cardiomyopathy in children. Patients with severe cardiac dysfunction are thought to be at risk of sudden cardiac arrest (SCA). After diagnosis, a period of medical optimization is recommended before permanent implantable cardioverter-defibrillator (ICD) implantation. Wearable cardioverter-defibrillators (WCDs) provide an option for arrhythmia protection as an outpatient during this optimization. OBJECTIVE: The purpose of this study was to determine the strategy that optimizes cost and survival during medical optimization of a patient with DCM before ICD placement. METHODS: A Markov state transition model was constructed for the 3 clinical approaches to compare costs, clinical outcomes, and quality of life: (1) "Inpatient," (2) "Home-WCD," and (3) "Home-No WCD." Transitional probabilities, costs, and utility metrics were extracted from the existing literature. Cost-effectiveness was assessed comparing each paradigm's incremental cost-effectiveness ratio against a societal willingness-to-pay threshold of $50,000 per quality-adjusted life year. RESULTS: The cost-utility analysis illustrated that Home-WCD met the willingness-to-pay threshold with an incremental cost-effectiveness ratio of $20,103 per quality-adjusted life year and 4 mortalities prevented per 100 patients as compared with Home-No WCD. One-way sensitivity analyses demonstrated that Home-No WCD became the most cost-effective solution when the probability of SCA fell below 0.2% per week, the probability of SCA survival with a WCD fell below 9.8%, or the probability of SCA survival with Home-No WCD quadrupled from base-case assumptions. CONCLUSION: Based on the existing literature probabilities of SCA in pediatric patients with DCM undergoing medical optimization before ICD implantation, sending a patient home with a WCD may be a cost-effective strategy.

Can noninvasive testing identify benign patterns of suggested pre-excitation on electrocardiogram?

BACKGROUND: The presence of anterograde conduction through an accessory pathway (AP) has been linked to sudden cardiac death. Unfortunately, pre-excitation associated with classic pathways can be difficult to differentiate from benign APs such as nodofascicular fibers. OBJECTIVE: Identifying characteristics on electrocardiogram (ECG) and exercise that differentiate classic and benign AP connections in suggested pre-excitation patterns. METHODS: Retrospective review of patients presenting between 1995 and 2017 with ventricular pre-excitation on ECG, determined to have either typical left-lateral AP during electrophysiology study (EPS), or benign, or no AP determined by either transesophageal electrophysiology study (TEP), or EPS. RESULTS: A total of 96 patients were included, 14.2 years (4-24), 45% female, 90% Caucasian. Of these, 60 (63%) had a classic APs identified on EPS and 58 (97%) underwent successful ablation. Conversely, 36 (37%) had benign pathways identified. ECG findings differed between the groups: PR-interval 102 versus 120 ms (P < .0001), QRS-duration 110 versus 102 ms (P < .0001), QRS-axis 74 versus 59 degrees (P = .0005), and QRS onset to peak R/S in limb leads 64 versus 42 ms (P < .0001), and precordial leads 66 versus 46 ms (P < .0001). Change in QRS duration during exercise differed between the groups: 25 versus 2 ms (P < .0001) and ECG characteristics identified the presence of an AP with 97% sensitivity and 94% negative predictive value. CONCLUSION: Classic and benign APs exhibit different ECG characteristics, though clinical overlap does not allow for absolute differentiation. These data may help with risk stratification decision making though does not obviate the need for additional invasive testing.

Ventricular force-frequency relationships during biventricular or multisite pacing in congenital heart disease.

BACKGROUND: Traditional indices to evaluate biventricular (BiV) pacing are load dependent, fail to assess dynamic changes, and may not be appropriate in patients with congenital heart disease (CHD). We therefore measured the force-frequency relationship (FFR) using tissue Doppler-derived isovolumic acceleration (IVA) to assess the dynamic adaption of the myocardium and its variability with different ventricular pacing strategies. METHODS: This was a prospective pilot study of pediatric and young adult CHD patients with biventricular or multisite pacing systems. Color-coded myocardial velocities were recorded at the base of the systemic ventricular free wall. IVA was calculated at resting heart rate and with incremental pacing. FFR curves were obtained by plotting IVA against heart rate for different ventricular pacing strategies. RESULTS: Ten patients were included (mean: 22 ± 7 years). The FFR identified a best and worst ventricular pacing strategy for each patient, based on the AUC at baseline, submaximal, and peak heart rates (P < .001). However, there was no single best ventricular pacing strategy that was optimal for all patients. Additionally, the best ventricular pacing strategy often differed within the same patient at different heart rates. CONCLUSION: This novel assessment demonstrates a wide variability in optimal ventricular pacing strategy. These inherent differences may play a role in the unpredictable clinical response to BiV pacing in CHD, and emphasizes an individualized approach. Furthermore, the optimal ventricular pacing varies with heart rate within individuals, suggesting that rate-responsive ventricular pacing modulation may be required to optimize ventricular performance.

Characteristics and Outcomes of Pediatric Patients Who Undergo Placement of Implantable Cardioverter Defibrillators: Insights From the National Cardiovascular Data Registry.

Background Implantable cardioverter defibrillators (ICDs) are an important part of therapy for many patients, yet there is little data on population characteristics, complications, or system survival in pediatric patients. Methods A retrospective review of ICD recipients in the National Cardiovascular Data Registry ICD Registry was performed from 2010 to 2016. Patient characteristics and complications between pediatric (≤21 years) and adult populations (>21 years) were compared. Variables associated with complications and early device interventions within the pediatric cohort were evaluated using multivariate modeling. Results There were 562 209 total ICD implants, of which 3461 occurred in the pediatric cohort. Among the pediatric patients, 60% of implants were for primary prevention, and nonischemic cardiomyopathy was the most common underlying disease (60%). Over time, there was an increasing trend of both primary and secondary prevention ICD implantations ( P<0.05). Compared with adults, pediatric patients were more likely to have structural heart disease, hypertrophic cardiomyopathy, and channelopathy, and to receive a single-chamber device (all P<0.001). There was no difference in inhospital complications between the adult and pediatric cohorts (2.4% versus 2.6%, P=0.3). However, among the pediatric patients, lower weight, Ebstein anomaly, worse New York Heart Association class, dual chamber, and cardiac resynchronization therapy-defibrillator were associated with greater risk of complications. Although reintervention for generator replacement or upgrade was more common in adults, the time to reintervention was shorter in the pediatric cohort. Conclusions We observed an increasing trend in ICD device implantation among pediatric patients. The pediatric cohort had similar inhospital complication rates compared with adults but had a shorter time to reintervention.

The US Experience of the Wearable Cardioverter-Defibrillator in Pediatric Patients.

BACKGROUND: Certain pediatric patients are at risk for sudden cardiac death. The wearable cardioverter-defibrillator (WCD) can be used in clinical situations in which implantable cardioverter-defibrillator placement is not ideal. The objectives of the study are to examine the effectiveness, safety, and compliance of the WCD in the identification and treatment of life-threatening ventricular arrhythmias in pediatric patients. METHODS: All United States pediatric patients <18 years who wore a WCD, from 2009 to 2016 were retrospectively reviewed. RESULTS: In total, 455 patients were identified. The median age was 15 (3-17) years, median duration of WCD use was 33 (1-999) days and median patient wear time was 20.6 (0.3-23.8) hours per day. The population was divided into 2 groups: (1) patients with implantable cardioverter-defibrillator problem, n=63 and (2) patients with nonimplantable cardioverter-defibrillator problem, n=392. Wear time per day was >20 hours in both groups. Wear duration was shorter in the implantable cardioverter-defibrillator problem group, 26 days versus 35 days, P<0.05. There were 7 deaths (1.5%); all not wearing WCD at time of death. Eight patients (1.8%) received at least 1 WCD shock treatment. Of the 6 patients (1.3%) who had appropriate therapy, there were 7 episodes of either polymorphic ventricular tachycardia or ventricular fibrillation with a total of 13 treatments delivered. All episodes were successfully converted and the patients survived. CONCLUSIONS: The WCD has overall adequate compliance with appropriate wear times and wear durations in pediatric patients. The WCD is safe and effective in treating ventricular arrhythmias that can lead to sudden cardiac death in pediatric patients.