Treadmill exercise testing improves diagnostic accuracy in children with concealed congenital long QT syndrome.

BACKGROUND: Resting electrocardiogram (ECG) identification of long QT syndrome (LQTS) has limitations. Uncertainty exists on how to classify patients with borderline prolonged QT intervals. We tested if exercise testing could help serve to guide which children with borderline prolonged QT intervals may be gene positive for LQTS. METHODS: Pediatric patients (n = 139) were divided into three groups: Controls (n = 76), gene positive LQTS with borderline QTc (n = 21), and gene negative patients with borderline QTc (n = 42). Borderline QTc was defined between 440-470 (male) and 440-480 (female) ms. ECGs were recorded supine, sitting, and standing. Patients then underwent treadmill stress testing with Bruce protocol followed by a 9-minute recovery phase. RESULTS: Supine resting QTc, age, and Schwartz score for the three groups were: (a) gene positive: 446 ± 23 ms, 12.4 ± 3.4 years old, 3.2 ± 1.8; (b) gene negative: 445 ± 20 ms, 12.1 ± 2 years old, 2.0 ± 1.2; and (c) control: 400 ± 24 ms, 15.0 ± 3 years old. The three groups could be differentiated by their QTc response at two time points: standing and recovery phase at 6 minutes. Standing QTc ≥460 ms differentiated borderline prolonged QTc patients (gene positive and gene negative) from controls. Late recovery QTc ≥480 ms distinguished gene positive from gene negative patients. CONCLUSION: Exercise stress testing can be useful to identify children who are gene positive borderline LQTS from a normal population and gene negative borderline QTc children, allowing for selective gene testing in a higher risk group of patients with borderline QTc intervals and intermediate Schwartz scores.

Percutaneous epicardial placement of a prototype miniature pacemaker under direct visualization: An infant porcine chronic survival study.

INTRODUCTION: Pacemaker implantation in infants typically consists of surgical epicardial lead placement with an abdominal generator. Here, we describe the chronic performance of our minimally invasive prototype miniature pacemaker implanted under direct visualization in an immature porcine model. METHODS: Twelve piglets underwent miniature pacemaker implantation. A self-anchoring two-channel access port was inserted into a 1 cm incision in the subxiphoid space, and a thoracoscope was inserted into the main channel to visualize the thoracic cavity under insufflation. The pacemaker leadlet was inserted through a sheath via secondary channel and affixed against the epicardium using a helical side-biting electrode. The miniature pacemaker was tucked into the incision, which was sutured closed. Ventricular sensing, leadlet impedance, and capture thresholds were measured biweekly. A limited necropsy was performed after euthanasia. RESULTS: Nine piglets were followed for a median of 78 (IQR 52-82) days and gained 6.6 ± 3.2 kg. Three animals were censored from the analysis due to complications unrelated to the procedure. Capture thresholds rose above maximal output after a median of 67 (IQR 40-69) days. At termination, there was a significant decrease in R-wave amplitude (P = .03) and rise in capture thresholds at 0.4 ms (P = .01) and 1.0 ms pulse widths (P = .02). There was no significant change in leadlet impedance (P = .74). There were no wound infections. CONCLUSIONS: There were no infections following minimally invasive implantation of our prototype miniature pacemaker. Improvements to epicardial fixation are necessary to address diminished leadlet efficacy over time.

Effects of Dexmedetomidine on Myocardial Repolarization in Children Undergoing General Anesthesia: A Randomized Controlled Trial.

BACKGROUND: Dexmedetomidine is a highly selective α2-adrenergic agonist, which is increasingly used in pediatric anesthesia and intensive care. Potential adverse effects that have not been rigorously evaluated in children include its effects on myocardial repolarization, which is important given that the drug is listed as a possible risk factor for torsades de pointes. We investigated the effect of 3 different doses of dexmedetomidine on myocardial repolarization and transmural dispersion in children undergoing elective surgery with total IV anesthesia. METHODS: Sixty-four American Society of Anesthesiologists I-II children 3-10 years of age were randomized to receive dexmedetomidine 0.25 µg/kg, 0.5 µg/kg, 0.75 µg/kg, or 0 µg/kg (control), as a bolus administered over 60 seconds, after induction of anesthesia. Pre- and postintervention 12-lead electrocardiograms were recorded. The interval between the peak and the end of the electrocardiogram T wave (Tp-e; transmural dispersion) and heart rate-corrected QT intervals (myocardial repolarization) were measured by a pediatric electrophysiologist blinded to group allocation. Data were analyzed using an analysis of covariance regression model. The study was powered to detect a 25-millisecond difference in Tp-e. RESULTS: Forty-eight children completed the study, with data analyzed from 12 participants per group. There were no instances of dysrhythmias. Tp-e values were unaffected by dexmedetomidine administration at any of the studied doses (F = 0.09; P = .96). Mean (99% CI) within-group differences were all <2 milliseconds (-5 to 8). Postintervention, corrected QT interval increased in the control group, but decreased in some dexmedetomidine groups (F = 7.23; P < .001), specifically the dexmedetomidine 0.5 and 0.75 µg/kg doses. Within groups, the mean (99% CI) differences between pre- and postintervention corrected QT interval were 12.4 milliseconds (-5.8 to 30.6) in the control group, -9.0 milliseconds (-24.9 to 6.9) for dexmedetomidine 0.25 µg/kg, -18.6 milliseconds (-33.7 to -3.5) for dexmedetomidine 0.5 µg/kg, and -14.1 milliseconds (-27.4 to -0.8) for dexmedetomidine 0.75 µg/kg. CONCLUSIONS: Of the bolus doses of dexmedetomidine studied, none had an effect on Tp-e and the dexmedetomidine 0.5 and 0.75 µg/kg doses shortened corrected QT intervals when measured at 1 minute after dexmedetomidine bolus injection during total IV anesthesia. There is no evidence for an increased risk of torsades de pointes in this context.

Risk Factors for Development of Ectopic Atrial Tachycardia in Post-operative Congenital Heart Disease.

Ectopic atrial tachycardia (EAT) is common in surgically repaired congenital heart disease (CHD) and carries the potential for significant hemodynamic compromise. Our objective was to determine the incidence, and risk factors of EAT after CHD surgery. Prospective study of patients that underwent surgery for CHD from February to October 2016 was performed. Demographic, perioperative and electrophysiologic data were collected. Sustained EAT (> 30 s) was documented by telemetry or electrocardiogram and confirmed by a pediatric electrophysiologist. All patients were followed through index hospitalization. During the study period, 17/204 (8%) of patients developed EAT with median time-to-event of 14 days. 15/17 (88%) received anti-arrhythmic therapy for sustained EAT. By univariate analysis, younger age (5 vs. 284 days, P < .001), lower weight (3.2 vs. 7.5 kg, P < .001), single ventricle physiology (P = .05), longer cardiopulmonary bypass time (176 vs. 94 min, P < .001), need for delayed sternal closure (P < .001), and higher STAT category (P < .001) were associated with EAT. Incidence among single ventricle patients was 7/44 (16%), and of those 7/13 (54%) were < 30 days of age. Multivariable Cox regression analysis confirmed age at surgery < 30 days (hazard ratio = 11.7, P = .002) and use of milrinone (hazard ratio = 4.4, P = .007) as independent predictors of EAT. Post-operative EAT is frequent following surgery for CHD especially in neonates. Further study is warranted, specifically in the single ventricle population, given the high potential risk for arrhythmia-induced hemodynamic compromise in this vulnerable population.

The use of an integrated electroanatomic mapping system and intracardiac echocardiography to reduce radiation exposure in children and young adults undergoing ablation of supraventricular tachycardia.

AIMS: Non-fluoroscopic imaging (NFI) devices are increasingly used in ablations. The objective was to determine the utility of intracardiac echocardiography (ICE) in ablating paediatric supraventricular tachycardias (SVTs) and assess whether its integrated use with electroanatomic mapping (EAM) resulted in lower radiation exposure than use of EAM alone. METHODS AND RESULTS: Prospective, controlled, single-centre study of patients (pts) age ≥10 years, weight ≥35 kg, with SVT and normal cardiac anatomy. Patients were randomized to ICE + EAM (ICE) or EAM only (no ICE). Both had access to fluoroscopy as needed. Eighty-four pts were enroled (42 ICE, 42 no ICE). Median age was 15 years (range 10.4-23.7 years); 57% had accessory pathways, 42% atrioventricular nodal reentry tachycardia. There was no difference in radiation dose (9 mGy ICE vs. 23 mGy no ICE, P = 0.37) or fluoroscopy time (1.1 min ICE vs. 1.5 min no ICE, P = 0.38). Transseptal punctures were performed in 25 pts (16 ICE, 9 no ICE), with ICE reducing radiation (8 mGy ICE vs. 62 mGy no ICE, P = 0.002) and fluoroscopy time (1.1 min ICE vs. 4.5 min no ICE, P = 0.01). Zero fluoroscopy was achieved in 13 pts (15% of total, 5 ICE, 8 no ICE), and low-dose cases (<50 mGy) in 57 pts (68% of total, 33 ICE, 24 no ICE). Acute success was 95% for ICE, 88% for no ICE. CONCLUSION: Use of an integrated EAM/ICE system was no better than EAM alone in limiting radiation, but can be helpful for transseptal punctures. Given the low dose savings, use of ICE may be weighed against its financial cost. Low-fluoroscopy cases are performed in most NFI procedures.

Costs and benefits of targeted screening for causes of sudden cardiac death in children and adolescents.

BACKGROUND: Sudden cardiac death in children is a rare but devastating event. Experts have debated the merits of community-based screening programs using an ECG and targeting 2 potential high-risk groups: school-aged children initiating stimulant medications to treat attention-deficit/hyperactivity disorder and adolescents participating in sports. METHODS AND RESULTS: Simulation models incorporating detailed prevalence, sensitivity and specificity, and treatment algorithms were built to determine the cost-effectiveness of targeted sudden cardiac death screening. Clinical care algorithms were constructed for asymptomatic children initiating stimulants for attention-deficit/hyperactivity disorder (8 years of age) or participating in sports (14 years of age) and presenting with a positive ECG finding suggestive of 1 of the 3 most common pediatric disorders causing sudden cardiac death and identifiable by ECG. Information to develop simulation model assumptions was drawn from the existing literature, Medicaid fees, and expert judgment. Sensitivity analyses examined parameter ranges to identify influential sources of uncertainty. Outcomes included costs and lost life-years caused by condition-related mortality. Our models estimate that screening for all 3 conditions simultaneously would reduce sudden death risk by 3.6 to 7.5 × 10(-5) with projected life expectancy increases of 0.8 to 1.6 days per screened individual. The incremental cost-effectiveness of screening is $91,000 to $204,000 per life-year. Sensitivity analysis showed that assumed disease prevalence, baseline mortality, and the relative risk of mortality resulting from stimulant medication use and sports participation had the greatest impact on estimated cost-effectiveness. CONCLUSION: Results based on assumptions favoring sudden cardiac death screening indicated that its cost is high relative to its health benefits.

The electrophysiological characteristics of accessory pathways in pediatric patients with intermittent preexcitation.

BACKGROUND: Accessory pathways (APs) with intermittent preexcitation (IPX) are thought to be of lower risk, but there are reports of IPX patients presenting with rapidly conducted atrial fibrillation. METHODS: Retrospective study performed on patients with preexcitation who underwent an electro-physiological study (EPS). IPX was defined as loss of the delta wave on electrocardiogram prior to EPS. Patients with IPX were compared with those with persistent preexcitation (PPX) or suppression of the delta wave on exercise test (IPX-ET). Congenital heart disease and prior ablations were excluded. RESULTS: Of 328 patients with preexcitation, 41 (12.5%) had IPX. Patients with IPX or PPX were similar in age (12.9 years vs 13.0 years, P = 0.8) and AP location (left-sided 54% vs 50%, P = 0.7; septal 32% vs 35%, P = 0.4). Testing on isoproterenol was performed in 17 (41%) IPX and 41 (14%) PPX patients. Although IPX patients had a longer median refractory period compared to PPX patients (340 ms vs 310 ms, P = 0.001), the incidence of APs with refractory periods ≤250 ms was similar (10% vs 12%, P = 1.0). Exercise tests were performed on 208 patients and 24 (12%) had IPX-ET. Compared with IPX patients, IPX-ET had similar median AP refractory periods (320 ms, P = 0.4) and incidence of APs with refractory periods ≤250 ms (13%, P = 1.0). CONCLUSION: Patients with IPX had longer AP refractory periods than those with PPX, but the incidence of pathways with refractory periods ≤250 ms was not significantly different. The finding of IPX on a baseline electrocardiogram does not rule out potentially high-risk pathways.

Leadless Pacemakers in Patients with Congenital Heart Disease.

Transcatheter leadless pacemakers have benefits in congenital heart disease because they eliminate the risks of lead malfunction, venous occlusions, and pocket complications. This newest pacemaker's utility in this population has been limited by the large sheath and delivery system, need for atrioventricular synchronous pacing, lack of explantation options, and possible lack of adequate access to the subpulmonary ventricle. With careful planning, leadless pacing can be successfully performed in these patients. Consideration of nonfemoral access, alternative implant sites to avoid myocardial scar or prosthetic material, anticoagulation for patients with persistent intracardiac shunts or systemic ventricular implantation, and operator experience are critical.

Transcatheter Leadless Pacing in Children: A PACES Collaborative Study in the Real-World Setting.

BACKGROUND: Transcatheter Leadless Pacemakers (TLP) are a safe and effective option for adults with pacing indications. These devices may be an alternative in pediatric patients and patients with congenital heart disease for whom repeated sternotomies, thoracotomies, or transvenous systems are unfavorable. However, exemption of children from clinical trials has created uncertainty over the indications, efficacy, and safety of TLP in the pediatric population. The objectives of this study are to evaluate clinical indications, procedural characteristics, electrical performance, and outcomes of TLP implantation in children. METHODS: Retrospective data were collected from patients enrolled in the Pediatric and Congenital Electrophysiology Society TLP registry involving 15 centers. Patients ≤21 years of age who underwent Micra (Medtronic Inc, Minneapolis, MN) TLP implantation and had follow-up of ≥1 week were included in the study. RESULTS: The device was successfully implanted in 62 of 63 registry patients (98%) at a mean age of 15±4.1 years and included 20 (32%) patients with congenital heart disease. The mean body weight at TLP implantation was 55±19 kg and included 8 patients ≤8 years of age and ≤30 kg in weight. TLP was implanted by femoral (n=55, 87%) and internal jugular (n=8, 12.6%) venous approaches. During a mean follow-up period of 9.5±5.3 months, there were 10 (16%) complications including one cardiac perforation/pericardial effusion, one nonocclusive femoral venous thrombus, and one retrieval and replacement of TLP due to high thresholds. There were no deaths, TLP infections, or device embolizations. Electrical parameters, including capture thresholds, R wave sensing, and pacing impedances, remained stable. CONCLUSIONS: Initial results from the Pediatric and Congenital Electrophysiology Society TLP registry demonstrated a high level of successful Micra device implants via femoral and internal venous jugular approaches with stable electrical parameters and infrequent major complications. Long-term prospective data are needed to confirm the reproducibility of these initial findings.

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.

Management and outcomes of atrial fibrillation in 241 healthy children and young adults: Revisiting "lone" atrial fibrillation-A multi-institutional PACES collaborative study.

BACKGROUND: Atrial fibrillation (AF) in healthy children and young adults is rare. Risk of recurrence and treatment efficacy are not well defined. OBJECTIVE: The purpose of this study was to assess recurrence patterns and treatment efficacy in AF. METHODS: A retrospective multicenter cohort study including 13 congenital heart centers was facilitated by the Pediatric & Congenital Electrophysiology Society (PACES). Patients ≤21 years of age with documented AF from January 2004 to December 2018 were included. Demographics, family and clinical history, medications, electrophysiological study parameters, and outcomes related to the treatment of AF were recorded and analyzed. Patients with contributory diseases were excluded. RESULTS: In 241 subjects (83% male; mean age at onset 16 years), AF recurred in 94 patients (39%) during 2.1 ± 2.6 years of follow-up. In multivariable analysis, predictors of AF recurrence were family history in a first-degree relative <50 years of age (odds ratio [OR] 1.9; P = .047) and longer PR interval in sinus rhythm (OR 1.1 per 10 ms; P = .037). AF recurrence was similar whether patients began no treatment (39/125 [31%]), began daily antiarrhythmic therapy (24/63 [38%]), or had an ablation at any time (14/53 [26%]; P = .39). Ablating non-AF substrate with supraventricular tachycardia improved freedom from AF recurrence (P = .013). CONCLUSION: Recurrence of AF in the pediatric population is common, and the incidence of recurrence was not impacted by "no treatment," "medication only," or "ablation" treatment strategy. Ablation of pathways and other reentrant targets was the only intervention that decreased AF recurrence in children and young adults.

Minimally invasive percutaneous epicardial placement of a prototype miniature pacemaker with a leadlet under direct visualization: A feasibility study in an infant porcine model.

BACKGROUND: Pacemaker implantation in infants is limited to epicardial lead placement and an abdominal generator pocket. We propose a minimally invasive solution using a prototype miniature pacemaker with a steroid-eluting leadlet that can affix against the epicardium under thoracoscopy. OBJECTIVE: The purpose of this study was to evaluate the safety and feasibility of acute implantation of a prototype miniature pacemaker in an infant porcine model. METHODS: A self-anchoring 2-channel access port was inserted into a 1-cm incision left of the subxiphoid space. A rigid thoracoscope with variable viewing angle was inserted through the main channel to visualize the heart under insufflation. An 18-G needle through the second channel accessed the pericardial space, which was secured with a 7-F sheath. The leadlet was affixed against the epicardium using a distal helical side-biting electrode. The sheath, thoracoscope, and port were removed, and the pacemaker was tucked into the incision. Ventricular sensing, lead impedances, and capture thresholds were measured. RESULTS: Twelve piglets (weight 4.8 ± 1.9 kg) had successful device implantation. The median time from incision to leadlet fixation was 21 minutes (interquartile range [IQR] 18-31 minutes). The median lead impedance was 510 Ω (IQR 495-620 Ω). The median R-wave amplitude was 5.7 mV (IQR 4.2-7.0 mV). The median capture threshold was 1.63 V (IQR 1.32-2.97 V) at 0.4 ms pulse width and 1.50 V (IQR 1.16-2.38 V) at 1.0 ms pulse width. There were no complications. CONCLUSION: Minimally invasive epicardial placement of a prototype miniature pacemaker under thoracoscopy was safe and avoided open chest surgery and creation of an abdominal generator pocket.

Sudden Cardiac Death in Children and Adolescents.

Sudden cardiac death (SCD) is a rare but devastating event in children and adolescents. Etiologies include congenital heart disease, cardiomyopathies, primary arrhythmia syndromes, and miscellaneous conditions. Challenges in the diagnosis and prevention of SCD in the young are reviewed.

Ebstein Anomaly.

Ebstein anomaly is a rare form of congenital heart disease with a uniquely high prevalence of arrhythmias. The most prevalent arrhythmia mechanisms are intrinsic to the underlying embryologic defects and may manifest at any stage. Current electrophysiological and surgical strategies are well equipped to address these arrhythmia mechanisms, yet despite available technology and a robust understanding of the mechanisms, these cases remain challenging. Surgical techniques that render arrhythmia substrates unreachable mandate comprehensive presurgical electrophysiological assessment and potential ablation. As the population ages, the need to address atrial fibrillation management and risk stratification for sudden cardiac death becomes ever more pertinent.

Genetic Insurance Discrimination in Sudden Arrhythmia Death Syndromes: Empirical Evidence From a Cross-Sectional Survey in North America.

BACKGROUND: There is virtually no information assessing the insurability of families affected with Sudden Arrhythmia Death Syndromes (SADS) for the determination of the nonclinical implications of genetic screening. It is important to identify the barriers and challenges faced by families as a result of genetic screening for SADS to enable equitable access to insurance coverage. METHODS AND RESULTS: To explore the insurance coverage experiences of SADS-affected families, we administered a cross-sectional online survey across North America from April 28, 2012 to November 13, 2013. Participants included individuals with a SADS diagnosis and their relatives who have applied for insurance (health, life, travel, and disability) or have existing insurance coverage. Of 202 participants, 92% had a SADS diagnosis (92%) as either a proband (50%) or an affected relative (42%); 8% of participants were unaffected family members of a proband; and genetic confirmation was reported by 73%. Of the 54% of SADS respondents who applied for insurance, 60% were rejected by insurers. The preexisting SADS diagnosis was the major reason reported for rejection (57%). Most respondents (80%) had insurance coverage through a spouse/parent plan at the time of diagnosis; 14% experienced a subsequent negative effect on coverage. Thirty-nine percent of affected SADS respondents reported an increase in insurance premium rates. CONCLUSIONS: Increased genetic testing has negatively impacted insurability for SADS patients and affected family members. The challenges in obtaining life and health insurance are mainly because of the preexisting condition, even in the presence of protective laws in the United States.

Mechanism and ablation of arrhythmia following total cavopulmonary connection.

BACKGROUND: The ability to identify and ablate different arrhythmia mechanisms after the total cavopulmonary connection has not been studied in detail. METHODS AND RESULTS: After obtaining Institutional Review Board approval according to institutional guidelines, consecutive patients after a total cavopulmonary connection undergoing electrophysiology study over a 6-year period were included (2006-2012). Arrhythmia mechanism was determined, and the procedural outcome was defined as complete, partial success, or failure. A 12-point arrhythmia severity score was calculated for each patient at baseline and on follow-up. Fifty-seven procedures were performed on 52 patients (18.4 ± 11.8 years; 53.0 ± 27.2 kg). Access to the pulmonary venous atrium was necessary in 33 procedures, via fenestration (16) or transbaffle puncture (17), and in 2 cases, an additional retrograde approach was used. In total, 80 arrhythmias were identified in 47 cases: macroreentrant (n = 25) or focal atrial tachycardia (n = 8), atrioventricular nodal reentry tachycardia (n = 13), reentry via an accessory pathway (n = 4) or via twin atrioventricular nodes (n = 4), ventricular tachycardia (n = 5), and undefined atrial tachycardia (n = 21). Procedural outcome in 32 patients who underwent ablation was complete success (n = 25), partial success (n = 3), failure (n = 3), or empirical ablation (n = 1). After successful ablation, there was a significant decrease in arrhythmia score over 18.2 (4-32) months follow-up, with a sustained trend even in the face of arrhythmia recurrence (50%). CONCLUSIONS: Arrhythmia mechanism post total cavopulmonary connection is highly varied, encompassing simple and more complex substrates, documentation of which facilitates a strategic approach to invasive arrhythmia management. Despite the anatomic limitations, successful and clinically meaningful ablation is possible.

Utility of preoperative electrophysiologic studies in patients with Ebstein's anomaly undergoing the Cone procedure.

BACKGROUND: Ebstein's anomaly is associated with a high incidence of atrial and ventricular arrhythmias. The Cone procedure has become an effective hemodynamic intervention for this malformation. In response to two late postoperative sudden deaths in our early institutional Cone experience, a standardized plan for aggressive rhythm evaluation was instituted, including preoperative electrophysiologic studies (EPS) and intraoperative implantable loop recorder (ILR) placement. OBJECTIVE: The purpose of this study was to measure the yield of this diagnostic protocol for patients with Ebstein's anomaly undergoing surgical repair and to describe its influence on patient management. METHODS: All patients at Boston Children's Hospital with Ebstein's anomaly who underwent the Cone procedure from December 2006 to September 2012 were reviewed. Pre- and postoperative arrhythmias and therapies were documented. For patients who underwent preoperative EPS, all arrhythmia substrates and interventions were recorded. RESULTS: A total of 74 patients were included, 42 of whom underwent preoperative EPS. Significant findings were documented during EPS in 29 of the 42 patients (69%), including eight patients with no prior suspicion of arrhythmias. Seventeen patients had successful catheter ablation during EPS, and EPS data guided one or more intraoperative rhythm interventions for the remainder. During follow-up, diagnostic yield from ILR was low. Since implementing a more aggressive diagnostic strategy, no further sudden deaths have occurred. CONCLUSION: Preoperative EPS has a high diagnostic and therapeutic yield in patients with Ebstein's anomaly undergoing the Cone operation. It is reasonable to recommend EPS as a routine preoperative test for this population.