Kara Motonaga

All Publications

• Multicenter Study of Survival Benefit of Cardiac Resynchronization Therapy in Pediatric and Congenital Heart Disease. JACC. Clinical electrophysiology Chubb, H., Mah, D. Y., Shah, M., Lin, K. Y., Peng, D. M., Hale, B. W., May, L., Etheridge, S., Goodyer, W., Ceresnak, S. R., Motonaga, K. S., Rosenthal, D. N., Almond, C. S., McElhinney, D. B., Dubin, A. M. 2023

  Abstract

  BACKGROUND: Evidence for the efficacy of cardiac resynchronization therapy (CRT) in pediatric and congenital heart disease (CHD) has been limited to surrogate outcomes.OBJECTIVES: This study aimed to assess the impact of CRT upon the risk of transplantation or death in a retrospective, high-risk, controlled cohort at 5 quaternary referral centers.METHODS: Both CRT patients and control patients were<21 years of age or had CHD; had systemic ventricular ejection fraction<45%; symptomatic heart failure; and significant electrical dyssynchrony (QRS duration z score >3 or single-site ventricular pacing >40%) at enrollment. Patients with CRT were matched with control patients via 1:1 propensity score matching. CRT patients were enrolled at CRT implantation; control patients were enrolled at the outpatient clinical encounter where inclusion criteria were first met. The primary endpoint was transplantation or death.RESULTS: In total, 324 control patients and 167 CRT recipients were identified. Mean follow-up was 4.2 ± 3.7 years. Upon propensity score matching, 139 closely matched pairs were identified (20 baseline indices). Of the 139 matched pairs, 52 (37.0%) control patients and 31 (22.0%) CRT recipients reached the primary endpoint. On both unadjusted and multivariable Cox regression analysis, the risk reduction associated with CRT for the primary endpoint was significant (HR: 0.40; 95%CI: 0.25-0.64; P< 0.001; and HR: 0.44; 95%CI: 0.28-0.71; P=0.001, respectively). On longitudinal assessment, the CRT group had significantly improved systemic ventricular ejection fraction (P< 0.001) and shorter QRS duration (P=0.015), sustained to 5 years.CONCLUSIONS: In pediatric and CHD patients with symptomatic systolic heart failure and electrical dyssynchrony, CRT was associated with improved heart transplantation-free survival.

  View details for DOI 10.1016/j.jacep.2023.11.008

  View details for PubMedID 38206260

• Utility of smart watches for identifying arrhythmias in children. Communications medicine Zahedivash, A., Chubb, H., Giacone, H., Boramanand, N. K., Dubin, A. M., Trela, A., Lencioni, E., Motonaga, K. S., Goodyer, W., Navarre, B., Ravi, V., Schmiedmayer, P., Bikia, V., Aalami, O., Ling, X. B., Perez, M., Ceresnak, S. R. 2023; 3 (1): 167

  Abstract

  Arrhythmia symptoms are frequent complaints in children and often require a pediatric cardiology evaluation. Data regarding the clinical utility of wearable technologies are limited in children. We hypothesize that an Apple Watch can capture arrhythmias in children.We present an analysis of patients ≤18 years-of-age who had signs of an arrhythmia documented by an Apple Watch. We include patients evaluated at our center over a 4-year-period and highlight those receiving a formal arrhythmia diagnosis. We evaluate the role of the Apple Watch in arrhythmia diagnosis, the results of other ambulatory cardiac monitoring studies, and findings of any EP studies.We identify 145 electronic-medical-record identifications of Apple Watch, and find arrhythmias confirmed in 41 patients (28%) [mean age 13.8 ± 3.2 years]. The arrythmias include: 36 SVT (88%), 3 VT (7%), 1 heart block (2.5%) and wide 1 complex tachycardia (2.5%). We show that invasive EP study confirmed diagnosis in 34 of the 36 patients (94%) with SVT (2 non-inducible). We find that the Apple Watch helped prompt a workup resulting in a new arrhythmia diagnosis for 29 patients (71%). We note traditional ambulatory cardiac monitors were worn by 35 patients (85%), which did not detect arrhythmias in 10 patients (29%). In 73 patients who used an Apple Watch for recreational or self-directed heart rate monitoring, 18 (25%) sought care due to device findings without any arrhythmias identified.We demonstrate that the Apple Watch can record arrhythmia events in children, including events not identified on traditionally used ambulatory monitors.

  View details for DOI 10.1038/s43856-023-00392-9

  View details for PubMedID 38092993

  View details for PubMedCentralID 4937287

• Outcomes After Development of Ventricular Arrhythmias in Single Ventricular Heart Disease Patients With Fontan Palliation. Circulation. Arrhythmia and electrophysiology Giacone, H. M., Chubb, H., Dubin, A. M., Motonaga, K. S., Ceresnak, S. R., Goodyer, W. R., Hanish, D., Trela, A. V., Boramanand, N., Lencioni, E., Boothroyd, D., Graber-Naidich, A., Wright, G., Haeffele, C., Hollander, S. A., McElhinney, D. B., Ma, M., Hanley, F. L., Chen, S. 2023: e011143

  Abstract

  With the advent of more intensive rhythm monitoring strategies, ventricular arrhythmias (VAs) are increasingly detected in Fontan patients. However, the prognostic implications of VA are poorly understood. We assessed the incidence of VA in Fontan patients and the implications on transplant-free survival.Medical records of Fontan patients seen at a single center between 2002 and 2019 were reviewed to identify post-Fontan VA (nonsustained ventricular tachycardia >4 beats or sustained >30 seconds). Patients with preFontan VA were excluded. Hemodynamically unstable VA was defined as malignant VA. The primary outcome was death or heart transplantation. Death with censoring at transplant was a secondary outcome.Of 431 Fontan patients, transplant-free survival was 82% at 15 years post-Fontan with 64 (15%) meeting primary outcome of either death (n=16, 3.7%), at a median 4.6 (0.4-10.2) years post-Fontan, or transplant (n=48, 11%), at a median of 11.1 (5.9-16.2) years post-Fontan. Forty-eight (11%) patients were diagnosed with VA (90% nonsustained ventricular tachycardia, 10% sustained ventricular tachycardia). Malignant VA (n=9, 2.0%) was associated with younger age, worse systolic function, and valvular regurgitation. Risk for VA increased with time from Fontan, 2.4% at 10 years to 19% at 20 years. History of Stage 1 surgery with right ventricular to pulmonary artery conduit and older age at Fontan were significant risk factors for VA. VA was strongly associated with an increased risk of transplant or death (HR, 9.2 [95% CI, 4.5-18.7]; P<0.001), with a transplant-free survival of 48% at 5-year post-VA diagnosis.Ventricular arrhythmias occurred in 11% of Fontan patients and was highly associated with transplant or death, with a transplant-free survival of <50% at 5-year post-VA diagnosis. Risk factors for VA included older age at Fontan and history of right ventricular to pulmonary artery conduit. A diagnosis of VA in Fontan patients should prompt increased clinical surveillance.

  View details for DOI 10.1161/CIRCEP.122.011143

  View details for PubMedID 37254747

• UTILITY OF THE APPLE WATCH (R) FOR IDENTIFYING ARRHYTHMIAS IN CHILDREN Zahedivash, A., Chubb, H., Giacone, H., Boramanand, N., Dubin, A., Trela, A., Lencioni, E., Motonaga, K., Goodyer, W., Ceresnak, S. R. ELSEVIER SCIENCE INC. 2023: 1563

  View details for Web of Science ID 000990866101575

• A Global Look to the Future of Pediatric Electrophysiology. JACC. Clinical electrophysiology Cohen, M. I., Dubin, A. M., Webster, G., Bar-Cohen, Y., Motonaga, K. S., Kriebel, T., Sanatani, S., Rosenthal, E., Moltedo, J., Yoshimoto, J., Davis, A., Van Hare, G. F., Etheridge, S. P. 2022; 8 (12): 1599-1602

  View details for DOI 10.1016/j.jacep.2022.09.015

  View details for PubMedID 36543515

• The development and efficacy of a paediatric cardiology fellowship online preparatory course. Cardiology in the young Motonaga, K. S., Sacks, L., Olson, I., Balasubramanian, S., Chen, S., Peng, L., Feinstein, J. A., Silverman, N. H., Hanley, F. L., Axelrod, D. M., Krawczeski, C. D., Arunamata, A., Kwiatkowski, D. M., Ceresnak, S. R. 2022: 1-6

  Abstract

  BACKGROUND: The transition from residency to paediatric cardiology fellowship is challenging due to the new knowledge and technical skills required. Online learning can be an effective didactic modality that can be widely accessed by trainees. We sought to evaluate the effectiveness of a paediatric cardiology Fellowship Online Preparatory Course prior to the start of fellowship.METHODS: The Online Preparatory Course contained 18 online learning modules covering basic concepts in anatomy, auscultation, echocardiography, catheterisation, cardiovascular intensive care, electrophysiology, pulmonary hypertension, heart failure, and cardiac surgery. Each online learning module included an instructional video with pre-and post-video tests. Participants completed pre- and post-Online Preparatory Course knowledge-based exams and surveys. Pre- and post-Online Preparatory Course survey and knowledge-based examination results were compared via Wilcoxon sign and paired t-tests.RESULTS: 151 incoming paediatric cardiology fellows from programmes across the USA participated in the 3 months prior to starting fellowship training between 2017 and 2019. There was significant improvement between pre- and post-video test scores for all 18 online learning modules. There was also significant improvement between pre- and post-Online Preparatory Course exam scores (PRE 43.6 ± 11% versus POST 60.3 ± 10%, p < 0.001). Comparing pre- and post-Online Preparatory Course surveys, there was a statistically significant improvement in the participants' comfort level in 35 of 36 (97%) assessment areas. Nearly all participants (98%) agreed or strongly agreed that the Online Preparatory Course was a valuable learning experience and helped alleviate some anxieties (77% agreed or strongly agreed) related to starting fellowship.CONCLUSION: An Online Preparatory Course prior to starting fellowship can provide a foundation of knowledge, decrease anxiety, and serve as an effective educational springboard for paediatric cardiology fellows.

  View details for DOI 10.1017/S1047951122003626

  View details for PubMedID 36440543

• 2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Executive Summary. Annals of pediatric cardiology Silka, M. J., Shah, M. J., Silva, J. N., Balaji, S., Beach, C. M., Benjamin, M. N., Berul, C. I., Cannon, B., Cecchin, F., Cohen, M. I., Dalal, A. S., Dechert, B. E., Foster, A., Gebauer, R., Corcia, M. C., Kannankeril, P. J., Karpawich, P. P., Kim, J. J., Krishna, M. R., Kubuš, P., LaPage, M. J., Mah, D. Y., Malloy-Walton, L., Miyazaki, A., Motonaga, K. S., Niu, M. C., Olen, M., Paul, T., Rosenthal, E., Saarel, E. V., Silvetti, M. S., Stephenson, E. A., Tan, R. B., Triedman, J., Bergen, N. H., Wackel, P. L. 2022; 15 (3): 323-346

  View details for DOI 10.4103/0974-2069.361245

  View details for PubMedID 36589659

  View details for PubMedCentralID PMC9802608

• The Rare Ventricular Arrhythmia Recurrence following Fibroma Resection: Can we predict who is at risk? Heart rhythm Motonaga, K. S. 2022

  View details for DOI 10.1016/j.hrthm.2022.10.025

  View details for PubMedID 36336301

• Ventricular arrhythmias following transcatheter pulmonary valve replacement with the harmony TPV25 device. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Taylor, A., Yang, J., Dubin, A., Chubb, M. H., Motonaga, K., Goodyer, W., Giacone, H., Peng, L., Romfh, A., McElhinney, D., Ceresnak, S. 2022

  Abstract

  BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) with the Harmony valve (Medtronic, Inc.) was recently approved to treat postoperative native outflow tract pulmonary regurgitation. While the 22mm Harmony valve Early Feasibility Study demonstrated ventricular tachycardia (VT) in only 5% of patients, little is known about ventricular arrhythmias after TPVR with the larger 25mm valve (TPV25).METHODS: A single center review was performed of patients with TPV25 implant from 2020 to 2021. Demographic, cardiac, procedural, and postimplant cardiac telemetry data were collected and compared between patients who did and did not have peri-implant ventricular arrhythmia.RESULTS: Thirty patients underwent TPV25 at a median age of 30 years. On postimplant telemetry, VT events were documented in 12 patients (40%); 11 nonsustained VT (NSVT) (median 3 episodes per patient and 6 beats per episode, maximum 157 episodes) and 1 sustained VT (3%), with Torsades de Pointes secondary to a short coupled premature ventricular contraction (PVC). VT events were associated with annular valve positioning (p<0.001) and increased postimplant PVC burden (p<0.0001), but there was no association between VT and other demongraphic, historical, or procedural factors. The frequency of NSVT events fell from 3/hfrom 0 to 12h postimplant to 0.5/hr from 12 to 24h (p<0.001).CONCLUSION: VT occurred commonly (40%) in the first 24h after TPV25 implant, with self-limited NSVT in 11 of 12 patients and 1 patient with cardiac arrest secondary to Torsades de Pointes. VT only occurred with annular valve positioning. Larger, longer-term studies are needed to determine risk factors for and natural history of post-TPVR VT.

  View details for DOI 10.1002/ccd.30393

  View details for PubMedID 36198126

• Post-Traumatic Stress Disorder in pediatric Implantable Cardioverter Defibrillator patients and their parents. Heart rhythm Schneider, L. M., Wong, J. J., Adams, R., Bates, B., Chen, S., Ceresnak, S. R., Danovsky, M., Hanisch, D., Motonaga, K. S., Restrepo, M., Shaw, R. J., Sears, S. F., Trela, A., Dubin, A. M., Hood, K. K. 2022

  Abstract

  An 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 PTSD.This study aimed to estimate the prevalence of PTSD in pediatric ICD patients and their parents and identify the factors associated with PTSD incidence.Pediatric participants with an ICD ages 8-21 years and parents of youth ages 0-21 years completed surveys that included demographics and PTSD measures. Pediatric participants completed additional psychosocial measures, such as anxiety and depression self-report questionnaires.Fifty youth (30% female) and 43 parents (70% female) completed the measures. Six youth (12%) met the screening criteria for a likely PTSD diagnosis, while 20 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=0.021), meet the clinical cutoff for depression (67% vs 16%, p=.005), and had higher shock anxiety (31.7 vs 17.9, p=0.003) than children without PTSD. Female gender (57% vs 23%, p=0.043) and patient depression (31% vs 5%, p=.042) were associated with PTSD among parents.Parents were found to be more likely to meet the criteria for PTSD than youth. Among youth, PTSD was associated with medical and psychosocial factors whereas PTSD among 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.

  View details for DOI 10.1016/j.hrthm.2022.06.025

  View details for PubMedID 35772698

• Risk Factors and Outcomes of Sudden Cardiac Arrest in Pediatric Heart Transplant Recipients. American heart journal Hollander, S. A., Barkoff, L., Giacone, H., Adamson, G. T., Kaufman, B. D., Motonaga, K. S., Dubin, A. M., Chubb, H. 2022

  Abstract

  Sudden cardiac arrest (SCA) is a prevailing cause of mortality after pediatric heart transplant (HT) but remains understudied. We analyzed the incidence, outcomes, and risk factors for SCA at our center.Retrospective review of all pediatric HT patients at our center from 1/1/2009-9/1/2021. SCA was defined as an abrupt loss of cardiac function requiring cardiopulmonary resuscitation and/or mechanical circulatory support (MCS). Events that occurred in the setting of limited resuscitative wishes, or while on MCS were excluded. Patient characteristics and risk factors were analyzed.Fourteen of 254 (6%) experienced SCA at a median of 3 (1, 4) years post-HT. Seven (50%) events occurred out-of-hospital. Eleven (79%) died from their initial event, 2 (18%) after failure to separate from extracorporeal membrane (ECMO). In univariate analysis, black race, younger donor age, prior acute cellular rejection (ACR) episode, pacemaker and/or ICD in place, and pre-mortem diagnosis of allograft vasculopathy were associated with SCA (P=0.003-0.02). In multivariable analysis, history of ACR, younger donor age, and black race retained significance. [OR=6.3, 95% CI: 1.6-25.4, P=0.01], [OR=0.9, 95% CI: 0.8-1, P=0.04], and [OR=7.3, 95% CI: 1.1-49.9, P=0.04], respectively. SCA occurred in 3 patients with a functioning ICD or pacemaker, which failed to restore a perfusing rhythm.SCA occurs relatively early after pediatric HT and is usually fatal. Half of events happen at home. Those who received younger donors, have a history of ACR, or are of black race are at increased risk. ICDs/pacemakers may offer limited protection.

  View details for DOI 10.1016/j.ahj.2022.06.003

  View details for PubMedID 35705134

• 2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Executive Summary ANNALS OF PEDIATRIC CARDIOLOGY Silka, M. J., Shah, M. J., Silva, J., Balaji, S., Beach, C. M., Benjamin, M. N., Berul, C., Cannon, B., Cecchin, F., Cohen, M., Dalal, A. S., Dechert, B. E., Foster, A., Gebauer, R., Corcia, M., Kannankeril, P. J., Karpawich, P. P., Kim, J. J., Krishna, M., Kubus, P., LaPage, M. J., Mah, D. Y., Malloy-Walton, L., Miyazaki, A., Motonaga, K. S., Niu, M. C., Olen, M., Paul, T., Rosenthal, E., Saarel, E., Silvetti, M., Stephenson, E. A., Tan, R. B., Triedman, J., Von Bergen, N. H., Wackel, P. L., Heart Rhythm Soc, Amer Coll Cardiology, Amer Heart Assoc, Assoc European Paediat Congenital, Asia Pacific Heart Rhythm Soc, Indian Heart Rhythm Soc, Latin Amer Heart Rhythm Soc 2022; 15 (3): 323-346

  View details for DOI 10.4103/0974-2069.361245

  View details for Web of Science ID 000892108400021

• VENTRICULAR ARRHYTHMIAS FOLLOWING TRANSCATHETER PULMONARY VALVE REPLACEMENT WITH THE HARMONY(C) TPV 25 DEVICE Taylor, A., Yang, J., Dubin, A., Chubb, M., Motonaga, K., Goodyer, W., Giacone, H., Peng, L. F., Romfh, A. W., McElhinney, D. B., Ceresnak, S. R. ELSEVIER SCIENCE INC. 2022: 1362

  View details for Web of Science ID 000781026601461

• Pediatric Arrhythmogenic Right Ventricular Cardiomyopathy: They May Be Small, But They Pack a Big Punch. JACC. Clinical electrophysiology Gonzalez Corcia, M. C., Motonaga, K. S. 2022; 8 (3): 319-321

  View details for DOI 10.1016/j.jacep.2021.09.014

  View details for PubMedID 35331426

• Implantable Cardioverter Defibrillators in Infants and Toddlers: Indications, Placement, Programming, and Outcomes. Circulation. Arrhythmia and electrophysiology Zahedivash, A., Hanisch, D., Dubin, A. M., Trela, A., Chubb, H., Motonaga, K., Goodyer, W., Maeda, K., Reinhartz, O., Ma, M., Martin, E., Ceresnak, S. 2022: CIRCEP121010557

  Abstract

  Limited data exist regarding implantable cardioverter defibrillator (ICD) usage in infants and toddlers. This study evaluates ICD placement indications, procedural techniques, programming strategies, and outcomes of ICDs in infants and toddlers.This is a single-center retrospective review of all patients ≤3 years old who received an ICD from 2009 to 2021.Fifteen patients received an ICD at an age of 1.2 years (interquartile range [IQR], 0.1-2.4; 12 [80%] women; weight, 8.2 kg [IQR, 4.2-12.6]) and were followed for a median of 4.28 years (IQR, 1.40-5.53) or 64.2 patient-years. ICDs were placed for secondary prevention in 12 patients (80%). Diagnoses included 8 long-QT syndromes (53%), 4 idiopathic ventricular tachycardias/ventricular fibrillations (VFs; 27%), 1 recurrent ventricular tachycardia with cardiomyopathy (7%), 1 VF with left ventricular noncompaction (7%), and 1 catecholaminergic polymorphic ventricular tachycardia (7%). All implants were epicardial, with a coil in the pericardial space. Intraoperative defibrillation safety testing was attempted in 11 patients (73%), with VF induced in 8 (53%). Successful restoration of sinus rhythm was achieved in all tested patients with a median of 9 (IQR, 7.3-11.3) J or 0.90 (IQR, 0.68-1.04) J/kg. Complications consisted of 1 postoperative chylothorax and 3 episodes of feeding intolerance. VF detection was programmed to 250 (IQR, 240-250) ms with first shock delivering 10 (IQR, 5-15) J or 1.1 (IQR, 0.8-1.4) J/kg. Three patients (20%) received appropriate shocks for ventricular tachycardia/VF. No patient received an inappropriate shock. There were 2 (13%) ventricular lead fractures (at 2.6 and 4.2 years post-implant), 1 (7%) pocket-site infection, and 2 (13%) generator exchanges. All patients were alive, and 1 patient (7%) received a heart transplant.ICDs can be safely and effectively placed for sudden death prevention in infants and toddlers with good midterm outcomes.

  View details for DOI 10.1161/CIRCEP.121.010557

  View details for PubMedID 35089800

• Mono- and Biallelic Protein-Truncating Variants in Alpha-Actinin 2 Cause Cardiomyopathy Through Distinct Mechanisms. Circulation. Genomic and precision medicine Lindholm, M. E., Jimenez-Morales, D., Zhu, H., Seo, K., Amar, D., Zhao, C., Raja, A., Madhvani, R., Abramowitz, S., Espenel, C., Sutton, S., Caleshu, C., Berry, G. J., Motonaga, K. S., Dunn, K., Platt, J., Ashley, E. A., Wheeler, M. T. 2021: CIRCGEN121003419

  Abstract

  BACKGROUND: ACTN2 (alpha-actinin 2) anchors actin within cardiac sarcomeres. The mechanisms linking ACTN2 mutations to myocardial disease phenotypes are unknown. Here, we characterize patients with novel ACTN2 mutations to reveal insights into the physiological function of ACTN2.METHODS: Patients harboring ACTN2 protein-truncating variants were identified using a custom mutation pipeline. In patient-derived iPSC-cardiomyocytes, we investigated transcriptional profiles using RNA sequencing, contractile properties using video-based edge detection, and cellular hypertrophy using immunohistochemistry. Structural changes were analyzed through electron microscopy. For mechanistic studies, we used coimmunoprecipitation for ACTN2, followed by mass-spectrometry to investigate protein-protein interaction, and protein tagging followed by confocal microscopy to investigate introduction of truncated ACTN2 into the sarcomeres.RESULTS: Patient-derived iPSC-cardiomyocytes were hypertrophic, displayed sarcomeric structural disarray, impaired contractility, and aberrant Ca2+-signaling. In heterozygous indel cells, the truncated protein incorporates into cardiac sarcomeres, leading to aberrant Z-disc ultrastructure. In homozygous stop-gain cells, affinity-purification mass-spectrometry reveals an intricate ACTN2 interactome with sarcomere and sarcolemma-associated proteins. Loss of the C-terminus of ACTN2 disrupts interaction with ACTN1 and GJA1, 2 sarcolemma-associated proteins, which may contribute to the clinical arrhythmic and relaxation defects. The causality of the stop-gain mutation was verified using CRISPR-Cas9 gene editing.CONCLUSIONS: Together, these data advance our understanding of the role of ACTN2 in the human heart and establish recessive inheritance of ACTN2 truncation as causative of disease.

  View details for DOI 10.1161/CIRCGEN.121.003419

  View details for PubMedID 34802252

• Identifying an Appropriate Endpoint for Cryoablation in Children with Atrioventricular Nodal Reentry Tachycardia: Is Residual Slow Pathway Conduction Associated with Recurrence? Heart rhythm Zook, N., DeBruler, K., Ceresnak, S., Motonaga, K., Goodyer, W., Trela, A., Dubin, A., Chubb, H. 2021

  Abstract

  BACKGROUND: Cryoablation is increasingly used to treat atrioventricular nodal reentry tachycardia (AVNRT) due to its safety profile. However, cryoablation may have higher recurrence than radiofrequency ablation (RFA) and the optimal procedural endpoint remains undefined.OBJECTIVE: The purpose of this study was to identify the association of cryoablation procedural endpoints with post-procedural AVNRT recurrence.METHODS: We performed a single-center, retrospective analysis of pediatric patients following successful first-time cryoablation for AVNRT between 1/1/2011 and 12/31/2019. Pre-ablation inducibility of AVNRT was recorded. Procedural endpoints, including slow pathway (SP) conduction (presence of jump or echo beats) with and without isoproterenol, were identified. Recurrence established from clinical notes and/or direct patient contact.RESULTS: Of 256 patients, 147(57%) were assessed on isoproterenol pre-cryoablation, and 171(47%) were assessed on isoproterenol post-cryoablation. Mean cryolesion time was 2586±1434 seconds. Following ablation, 104(41%) had some evidence of residual SP conduction. With median follow up time of 1.9[0.7-3.7] years, recurrence occurred in 14(5%) patients. Complete elimination of SP conduction (with and without isoproterenol) had a HR for recurrence of 1.26(95% CI 0.42-3.8, P=.68) on univariate analysis and 1.39(95% CI 0.36-5.4, P=.63) on multivariate analysis (including demographics, ablation time, 8mm cryocatheter and baseline inducibility).CONCLUSION: The observed AVNRT recurrence rate after cryoablation was comparable to RFA. The presence of residual SP conduction was not associated with recurrence. This suggests that jump or single echo beat may be an acceptable endpoint in AVNRT cryoablation.

  View details for DOI 10.1016/j.hrthm.2021.09.031

  View details for PubMedID 34601128

• A paediatric cardiology handbook for the digital age. Cardiology in the young Rochelson, E., Clark, B. C., Motonaga, K. S. 2021: 1-6

  Abstract

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

  View details for DOI 10.1017/S1047951121003061

  View details for PubMedID 34353409

• 2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients. Cardiology in the young Shah, M. J., Silka, M. J., Silva, J. A., Balaji, S., Beach, C., Benjamin, M., Berul, C., Cannon, B., Cecchin, F., Cohen, M., Dalal, A., Dechert, B., Foster, A., Gebauer, R., Gonzalez Corcia, M. C., Kannankeril, P., Karpawich, P., Kim, J., Krishna, M. R., Kubus, P., Malloy-Walton, L., LaPage, M., Mah, D., Miyazaki, A., Motonaga, K., Niu, M., Olen, M., Paul, T., Rosenthal, E., Saarel, E., Silvetti, M. S., Stephenson, E., Tan, R., Triedman, J., Von Bergen, N., Wackel, P. 2021: 1-104

  Abstract

  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 consenus 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 follow-up in pediatric patients.

  View details for DOI 10.1017/S1047951121003413

  View details for PubMedID 34338183

• 2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients. Indian pacing and electrophysiology journal Writing Committee Members, Shah, M. J., Silka, M. J., Silva, J. A., Balaji, S., Beach, C., Benjamin, M., Berul, C., Cannon, B., Cecchin, F., Cohen, M., Dalal, A., Dechert, B., Foster, A., Gebauer, R., Gonzalez Corcia, M. C., Kannankeril, P., Karpawich, P., Kim, J., Krishna, M. R., Kubus, P., Malloy-Walton, L., LaPage, M., Mah, D., Miyazaki, A., Motonaga, K., Niu, M., Olen, M., Paul, T., Rosenthal, E., Saarel, E., Silvetti, M. S., Stephenson, E., Tan, R., Triedman, J., Von Bergen, N., Wackel, P., Document Reviewers, Chang, P. M., Drago, F., Dubin, A., Kongpatanayothin, A., Moltedo, J. M., Nabar, A. A., Van Hare, G. F. 2021

  Abstract

  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 consenus 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 follow-up in pediatric patients.

  View details for DOI 10.1016/j.ipej.2021.07.005

  View details for PubMedID 34333141

• 2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Executive Summary. Indian pacing and electrophysiology journal Writing Committee Members, Silka, M. J., Shah, M. J., Silva, J. A., Balaji, S., Beach, C., Benjamin, M., Berul, C., Cannon, B., Cecchin, F., Cohen, M., Dalal, A., Dechert, B., Foster, A., Gebauer, R., Gonzalez Corcia, M. C., Kannankeril, P., Karpawich, P., Kim, J., Krishna, M. R., Kubus, P., Malloy-Walton, L., LaPage, M., Mah, D., Miyazaki, A., Motonaga, K., Niu, M., Olen, M., Paul, T., Rosenthal, E., Saarel, E., Silvetti, M. S., Stephenson, E., Tan, R., Triedman, J., Von Bergen, N., Wackel, P., Document Reviewers, Chang, P. M., Drago, F., Dubin, A., Kongpatanayothin, A., Moltedo, J. M., Nabar, A. A., Van Hare, G. F. 2021

  Abstract

  Guidelines for the implantation of cardiac implantable electronic devices (CIEDs) have evolved since publication of the initial ACC/AHA pacemaker guidelines in 1984.1 CIEDs have evolved to include novel forms of cardiac pacing, the development of implantable cardioverter-defibrillators (ICDs) and the introduction of devices for long term monitoring of heart rhythm and other physiologic parameters. In view of the increasing complexity of both devices and patients, practice guidelines, by necessity, have become increasingly specific. In 2018, the ACC/AHA/HRS published Guidelines on the Evaluation and Management of Patients with Bradycardia and Cardiac Conduction Delay,2 which were specific recommendations for patients >18 years of age. This age-specific threshold was established in view of the differing indications for CIEDs in young patients as well as size-specific technology factors. Therefore, the following document was developed to update and further delineate indications for the use and management of CIEDs in pediatric patients, defined as ≤21 years of age, with recognition that there is often overlap in the care of patents between 18 and 21 years of age. This document is an abbreviated expert consensus statement (ECS) intended to focus primarily on the indications for CIEDs in the setting of specific disease/diagnostic categories. This document will also provide guidance regarding the management of lead systems and follow-up evaluation for pediatric patients with CIEDs. The recommendations are presented in an abbreviated modular format, with each section including the complete table of recommendations along with a brief synopsis of supportive text and select references to provide some context for the recommendations. This document is not intended to provide an exhaustive discussion of the basis for each of the recommendations, which are further addressed in the comprehensive PACES-CIED document3, with further data easily accessible in electronic searches or textbooks.

  View details for DOI 10.1016/j.ipej.2021.07.006

  View details for PubMedID 34333142

• IMPLANTABLE CARDIOVERTER-DEFIBRILLATORS IN INFANTS AND TODDLERS: INDICATIONS, PLACEMENT, PROGRAMMING AND OUTCOMES Zahedivash, A., Hanisch, D., Dubin, A. M., Trela, A. V., Chubb, H., Motonaga, K., Goodyer, W., Maeda, K., Reinhartz, O., Ceresnak, S. ELSEVIER SCIENCE INC. 2021: 470

  View details for Web of Science ID 000647487500470

• 2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Executive Summary. Heart rhythm Silka, M. J., Shah, M. J., Silva, J. A., Balaji, S., Beach, C., Benjamin, M., Berul, C., Cannon, B., Cecchin, F., Cohen, M., Dalal, A., Dechert, B., Foster, A., Gebauer, R., Gonzalez Corcia, M. C., Kannankeril, P., Karpawich, P., Kim, J., Krishna, M. R., Kubuš, P., Malloy-Walton, L., LaPage, M., Mah, D., Miyazaki, A., Motonaga, K., Niu, M., Olen, M., Paul, T., Rosenthal, E., Saarel, E., Silvetti, M. S., Stephenson, E., Tan, R., Triedman, J., Von Bergen, N., Wackel, P. 2021

  View details for DOI 10.1016/j.hrthm.2021.07.051

  View details for PubMedID 34363987

• 2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients. Heart rhythm Shah, M. J., Silka, M. J., Silva, J. A., Balaji, S., Beach, C., Benjamin, M., Berul, C., Cannon, B., Cecchin, F., Cohen, M., Dalal, A., Dechert, B., Foster, A., Gebauer, R., Gonzalez Corcia, M. C., Kannankeril, P., Karpawich, P., Kim, J., Krishna, M. R., Kubuš, P., Malloy-Walton, L., LaPage, M., Mah, D., Miyazaki, A., Motonaga, K., Niu, M., Olen, M., Paul, T., Rosenthal, E., Saarel, E., Silvetti, M. S., Stephenson, E., Tan, R., Triedman, J., Von Bergen, N., Wackel, P. 2021

  Abstract

  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 consenus 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 follow-up in pediatric patients.

  View details for DOI 10.1016/j.hrthm.2021.07.038

  View details for PubMedID 34363988

• 2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Developed in collaboration with and endorsed by the Heart Rhythm Society (HRS), the American College of Cardiology (ACC), the American Heart Association (AHA), and the Association for European Paediatric and Congenital Cardiology (AEPC) Endorsed by the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). JACC. Clinical electrophysiology Shah, M. J., Silka, M. J., Silva, J. N., Balaji, S., Beach, C. M., Benjamin, M. N., Berul, C. I., Cannon, B., Cecchin, F., Cohen, M. I., Dalal, A. S., Dechert, B. E., Foster, A., Gebauer, R., Gonzalez Corcia, M. C., Kannankeril, P. J., Karpawich, P. P., Kim, J. J., Krishna, M. R., Kubuš, P., LaPage, M. J., Mah, D. Y., Malloy-Walton, L., Miyazaki, A., Motonaga, K. S., Niu, M. C., Olen, M., Paul, T., Rosenthal, E., Saarel, E. V., Silvetti, M. S., Stephenson, E. A., Tan, R. B., Triedman, J., Bergen, N. H., Wackel, P. L. 2021; 7 (11): 1437-1472

  Abstract

  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.

  View details for DOI 10.1016/j.jacep.2021.07.009

  View details for PubMedID 34794667

• 2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients: executive summary. Cardiology in the young Silka, M. J., Shah, M. J., Silva, J. N., Balaji, S., Beach, C. M., Benjamin, M. N., Berul, C. I., Cannon, B., Cecchin, F., Cohen, M. I., Dalal, A. S., Dechert, B. E., Foster, A., Gebauer, R., Gonzalez Corcia, M. C., Kannakeril, P. J., Karpawich, P. P., Kim, J. J., Krishna, M. R., Kubuš, P., LaPage, M. J., Mah, D. Y., Malloy-Walton, L., Miyazaki, A., Motonaga, K. S., Niu, M. C., Olen, M., Paul, T., Rosenthal, E., Saarel, E. V., Silvetti, M. S., Stephenson, E. A., Tan, R. B., Triedman, J., Von Bergen, N. H., Wackel, P. L. 2021; 31 (11): 1717-1737

  View details for DOI 10.1017/S1047951121003395

  View details for PubMedID 34796795

• Impact of Cardiac Resynchronization Therapy on Heart Transplant-Free Survival in Pediatric and Congenital Heart Disease Patients. Circulation. Arrhythmia and electrophysiology Chubb, H., Rosenthal, D. N., Almond, C. S., Ceresnak, S. R., Motonaga, K. S., Arunamata, A. A., Long, J., Trela, A. V., Hanisch, D., McElhinney, D. B., Dubin, A. M. 2020

  Abstract

  Background - Cardiac resynchronization therapy (CRT) studies in pediatric and/or congenital heart disease (CHD) patients have shown an improvement in ejection fraction and heart failure symptoms. However, a survival benefit of CRT in this population has not been established. This study aimed to evaluate the impact of CRT upon heart transplant-free survival in pediatric and CHD patients, using a propensity score-matched analysis. Methods - This single-center study compared CRT patients (implant date 2004-2017) and controls, matched by 1:1 propensity-score matching (PSM) using 21 comprehensive baseline indices for risk stratification. CRT patients were <21 years or had CHD; had systemic ventricular ejection fraction <;45%; symptomatic heart failure; and had significant electrical dyssynchrony, all prior to CRT implant. Controls were screened from non-selective imaging and ECG databases. Controls were retrospectively enrolled when they achieved the same inclusion criteria at an outpatient clinical encounter, within the same time period. Results - Of 133 patients who received CRT during the study period, 84 met all study inclusion criteria. 133 controls met all criteria at an outpatient encounter. Following PSM, 63 matched CRT-control pairs were identified with no significant difference between groups across all baseline indices. Heart transplant or death occurred in 12 (19%) PSM-CRT subjects and 37 (59%) PSM-controls with a median follow-up of 2.7 years (quartiles 0.8-6.1 years). CRT was associated with markedly reduced risk of heart transplant or death (hazard ratio 0.24 [95% CI 0.12-0.46], p<0.001). There was no CRT procedural mortality and one system infection at 54 months post-implant. Conclusions - In pediatric and CHD patients with symptomatic systolic heart failure and electrical dyssynchrony, CRT was associated with improved heart transplant-free survival.

  View details for DOI 10.1161/CIRCEP.119.007925

  View details for PubMedID 32202126

• Pectoral Nerve Blocks Decrease Postoperative Pain and Opioid Use After Pacemaker or Implantable Cardioverter-Defibrillator Placement in Children. Heart rhythm Yang, J. K., Char, D. S., Motonaga, K. S., Navaratnam, M., Dubin, A. M., Trela, A., Hanisch, D. G., McFadyen, G., Chubb, H., Goodyer, W., Ceresnak, S. R. 2020

  Abstract

  BACKGROUND: Pectoral nerve blocks (PECS) can reduce intra-procedural anesthetic requirements and postoperative pain. Little is known on the utility of PECS in reducing pain and narcotic use after pacemaker (PM) or implantable cardioverter defibrillator (ICD) placement in children.OBJECTIVE: 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 2015-2020 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 underwent PECS and those who underwent conventional local anesthetic (CONTROL).RESULTS: A total of 74 patients underwent PM or ICD placement with 20 patients (27%) undergoing 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%-CI 0.8-2.2) vs CONTROL 3.1 (95%-CI 2.7-3.5); P<0.001] and lower total opioid use [PECS 6.0 MME/m2 (95%-CI 3.4-8.6) vs CONTROL 15.0 MME/m2 (95%-CI 11.8-18.2); P=0.001] over the 24-hours post-implant.CONCLUSIONS: Pectoralis nerve blocks 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.

  View details for DOI 10.1016/j.hrthm.2020.03.009

  View details for PubMedID 32201270

• Can the 12-lead ECG distinguish RVOT from aortic cusp PVCs in pediatric patients? Pacing and clinical electrophysiology : PACE Clark, B. C., Ceresnak, S. R., Pass, R. H., Nappo, L., Sumihara, K., Dubin, A. M., Motonaga, K., Moak, J. P. 2020

  Abstract

  BACKGROUND: The ability to differentiate right ventricular outflow tract (RVOT) from coronary cusp (CC) site of origin (SOO) by 12-lead ECG in pediatric patients may impact efficacy and procedural time. The objective of this study was to predict RVOT versus CC SOO by ECG in pediatric patients.METHODS: Pediatric patients (<21 years) without structural heart disease with RVOT or CC premature ventricular contraction (PVC) ablations performed (2014 - 2018) were evaluated through multi-institution retrospective review. Demographics, ECG PVC parameters, ablation site, recurrence and repeat procedures were collected.RESULTS: 37 patients were evaluated (mean age 14.6 years, weight 60.6kg): 11 CC and 26 RVOT PVC SOO. CC PVCs were less likely to exhibit left bundle branch block (64vs. 100%, p=0.005), had larger R-wave amplitude in V1 (0.27vs. 0.11mV, p=0.03), larger R/S ratio in V1 (0.37vs. 0.09, p=0.003), and had precordial transition in V3 or earlier (73vs. 15%, p=0.002). A composite score was created with the following variables: isodiphasic or positive QRS in V1, R/S ratio in V1>0.05, S wave in V1<0.9mV and precordial transition at or before V3. Composite score ≥ 2 was associated with a CC SOO (OR 42.0, p=0.001, AUC 0.86).CONCLUSIONS: 12-lead ECG of PVCs from the CC was associated with larger V1 R-wave amplitude, larger R/S ratio in V1 and precordial transition at or before V3. A composite score may help predict PVC/VT arising from the CC. This article is protected by copyright. All rights reserved.

  View details for DOI 10.1111/pace.13885

  View details for PubMedID 32040211

• An exploratory assessment of pediatric patient and parent needs after implantable cardioverter defibrillator implant. Pacing and clinical electrophysiology : PACE Schneider, L. M., Wong, J. J., Trela, A. n., Hanisch, D. n., Shaw, R. J., Sears, S. F., Motonaga, K. S., Ceresnak, S. R., Hood, K. K., Dubin, A. M. 2020

  Abstract

  Placement of an ICD is often accompanied by psychological adjustment issues in pediatric patients and their parents. While anxiety, depression, and lowered QOL have been seen in these patients, no studies have investigated patient or parent reported needs. This exploratory study describes the needs of pediatric ICD patients and parents and assesses whether patient factors of age, gender, depression, and anxiety are associated with specific needs.ICD patients ages 8-21 and their parents completed a needs analysis survey assessing various domains of functioning. Patients also completed self-reported measures of depression and anxiety.Thirty-two patients (28% female) and their parents (72% mothers) completed the survey. Patients' most frequently endorsed needs involved educational issues: understanding their cardiac event/diagnosis (34%), medications (34%), and how the ICD would change their lifestyle (31%). Parents' most frequently endorsed needs involved family issues; almost half of parents (47%) were concerned about their children's frustration with their overprotectiveness and 28% were concerned with their child feeling depressed or anxious. Patients who reported feeling overprotected (12.1 ± 3.4 vs 17.4 ± 3.5 years, p = .001) were significantly younger than those who did not. Experiencing peer issues was more frequently endorsed by females than males (33% of females vs 4% of males, p = .026).ICD patients and parents endorsed markedly different needs. Patients focused on understanding their ICD, while parents were more focused on their children's emotional needs. Novel ways of educating patients about their device and clinic based screenings of emotional functioning may serve to meet these needs. This article is protected by copyright. All rights reserved.

  View details for DOI 10.1111/pace.13876

  View details for PubMedID 31971265

• Loss of Ventricular Pre-excitation During Non-invasive Testing Does Not Exclude High-Risk Accessory Pathways: A Multicenter Study of WPW in Children. Heart rhythm Escudero, C. A., Ceresnak, S. R., Collins, K. K., Pass, R. H., Aziz, P. F., Blaufox, A. D., Ortega, M. C., Cannon, B. C., Cohen, M. n., Dechert, B. E., Dubin, A. M., Motonaga, K. S., Epstein, M. R., Erickson, C. C., Fishberger, S. B., Gates, G. J., Capone, C. A., Nappo, L. n., Kertesz, N. J., Kim, J. J., Valdes, S. O., Kubuš, P. n., Law, I. H., Maldonado, J. n., Moore, J. P., Perry, J. C., Sanatani, S. n., Seslar, S. P., Shetty, I. n., Zimmerman, F. J., Skinner, J. R., Marcondes, L. n., Stephenson, E. A., Asakai, H. n., Tanel, R. E., Uzun, O. n., Etheridge, S. P., Janson, C. n. 2020

  Abstract

  Abrupt loss of ventricular pre-excitation on non-invasive evaluation, or non-persistent pre-excitation, in Wolff-Parkinson-White syndrome (WPW) is thought to indicate a low risk of life-threatening events.To compare accessory pathway (AP) characteristics and occurrences of sudden cardiac arrest (SCA) and rapidly conducted pre-excited atrial fibrillation (RC-AF) in patients with non-persistent and persistent pre-excitation.Patients ≤21 years with WPW and invasive electrophysiology study (EPS) data, SCA, or RC-AF were identified from multicenter databases. Non-persistent pre-excitation was defined as absence/sudden loss of pre-excitation on ECG, Holter, or exercise test. RC-AF was defined as clinical pre-excited atrial fibrillation with shortest pre-excited R-R interval (SPERRI) ≤250ms. AP effective refractory period (APERP), SPERRI at EPS (EPS-SPERRI), and shortest pre-excited paced cycle length (SPPCL) were collected. High-risk APs were defined as APERP, SPERRI, or SPPCL ≤250ms.Of 1589 patients, 244 (15%) had non-persistent pre-excitation and 1345 (85%) had persistent pre-excitation. There were no differences in sex (58 vs 60% male, p=0.49) or age (13.3±3.6 vs 13.1±3.9 years, p=0.43) between groups. Though APERP (344±76 vs 312±61ms, p<0.001), and SPPCL (394±123 vs 317±82ms, p<0.001) were longer in non-persistent versus persistent pre-excitation, there was no difference in EPS-SPERRI (331±71 vs 316±73ms, p=0.15). Non-persistent pre-excitation was associated with fewer high-risk APs (13 vs 23%, p<0.001) than persistent pre-excitation. Of 61 patients with SCA or RC-AF, 6 (10%) had non-persistent pre-excitation (3 SCA, 3 RC-AF).Non-persistent pre-excitation was associated with fewer high-risk APs, though it did not exclude risk of SCA or RC-AF in children with WPW.

  View details for DOI 10.1016/j.hrthm.2020.05.035

  View details for PubMedID 32497761

• Structural Abnormalities on Cardiac Magnetic Resonance Imaging in Patients With Catecholaminergic Polymorphic Ventricular Tachycardia. JACC. Clinical electrophysiology Gerber, D. A., Dubin, A. M., Ceresnak, S. R., Motonaga, K. S., Bussineau, M. n., Dunn, K. n., Caleshu, C. n., Shoemaker, M. B., Lubitz, S. A., Perez, M. V. 2020; 6 (6): 741–42

  View details for DOI 10.1016/j.jacep.2020.03.006

  View details for PubMedID 32553227

• Genotype Predicts Outcomes in Fetuses and Neonates With Severe Congenital Long QT Syndrome. JACC. Clinical electrophysiology Moore, J. P., Gallotti, R. G., Shannon, K. M., Bos, J. M., Sadeghi, E. n., Strasburger, J. F., Wakai, R. T., Horigome, H. n., Clur, S. A., Hill, A. C., Shah, M. J., Behere, S. n., Sarquella-Brugada, G. n., Czosek, R. n., Etheridge, S. P., Fischbach, P. n., Kannankeril, P. J., Motonaga, K. n., Landstrom, A. P., Williams, M. n., Patel, A. n., Dagradi, F. n., Tan, R. B., Stephenson, E. n., Krishna, M. R., Miyake, C. Y., Lee, M. E., Sanatani, S. n., Balaji, S. n., Young, M. L., Siddiqui, S. n., Schwartz, P. J., Shivkumar, K. n., Ackerman, M. J. 2020; 6 (12): 1561–70

  Abstract

  This study sought to determine the relationship between long QT syndrome (LQTS) subtype (LTQ1, LTQ2, LTQ3) and postnatal cardiac events (CEs).LQTS presenting with 2:1 atrioventricular block or torsades de pointes in the fetus and/or neonate has been associated with risk for major CEs, but overall outcomes and predictors remain unknown.A retrospective study involving 25 international centers evaluated the course of fetuses/newborns diagnosed with congenital LQTS and either 2:1 atrioventricular block or torsades de pointes. The primary outcomes were age at first CE after dismissal from the newborn hospitalization and death and/or cardiac transplantation during follow-up. CE was defined as aborted cardiac arrest, appropriate shock from implantable cardioverter-defibrillator, or sudden cardiac death.A total of 84 fetuses and/or neonates were identified with LQTS (12 as LQT1, 35 as LQT2, 37 as LQT3). Median gestational age at delivery was 37 weeks (interquartile range: 35 to 39 weeks) and age at hospital discharge was 3 weeks (interquartile range: 2 to 5 weeks). Fetal demise occurred in 2 and pre-discharge death in 1. Over a median of 5.2 years, there were 1 LQT1, 3 LQT2, and 23 LQT3 CEs (13 aborted cardiac arrests, 5 sudden cardiac deaths, and 9 appropriate shocks). One patient with LQT1 and 11 patients with LQT3 died or received cardiac transplant during follow-up. The only multivariate predictor of post-discharge CEs was LQT3 status (LQT3 vs. LQT2: hazard ratio: 8.4; 95% confidence interval: 2.6 to 38.9; p < 0.001), and LQT3, relative to LQT2, genotype predicted death and/or cardiac transplant (p < 0.001).In this large multicenter study, fetuses and/or neonates with LQT3 but not those with LQT1 or LQT2 presenting with severe arrhythmias were at high risk of not only frequent, but lethal CEs.

  View details for DOI 10.1016/j.jacep.2020.06.001

  View details for PubMedID 33213816

• QTc Prolongation and Risk of Torsades de Pointes in Hospitalized Pediatric Oncology Patients. The Journal of pediatrics Lim, T. R., Rangaswami, A. A., Dubin, A. M., Kapphahn, K. I., Sakarovitch, C., Long, J., Motonaga, K. S., Trela, T., Ceresnak, S. R. 2019

  Abstract

  OBJECTIVE: To evaluate the prevalence of torsades de pointes and to identify risk factors associated with QTc prolongation of ≥500milliseconds in hospitalized pediatric oncology patients. A QTc prolongation of ≥500milliseconds is associated with higher mortality in hospitalized adults but has not been demonstrated in pediatrics.STUDY DESIGN: A single-center, retrospective review of all hospitalized oncology patients ≤21years of age was performed from 2014 to 2016. Patients with long/short QT syndrome or a QRS interval of ≥120ms were excluded. Rapid response events were reviewed to determine the prevalence of torsades. In patients with ECGs for review, data were compared between patients with a QTc of <500 and≥500ms via logistic regression.RESULTS: There were 1934 hospitalized patients included. Rapid response events occurred in 90 patients (4.7%) with 2 torsades events (0.1%). There were 1412 electrocardiograms performed in 287 unique patients (10.6±6.3years of age; 43% female). The mean QTc was 448±31ms; 25 patients (8.7%) had ≥1 ECG with a QTc of ≥500ms. The prevalence of torsades was greater in patients with a QTc of ≥500ms (8% vs 0%; P<.01). In multivariate analysis, factors associated with a QTc of ≥500ms included female sex, (OR 2.95) and ≥2 QT-prolonging medications (OR, 2.95).CONCLUSIONS: The prevalence of torsades in hospitalized pediatric oncology patients was low (0.1%), although the risk was significantly greater in patients with a QTc of ≥500ms. Routine monitoring of electrocardiograms and electrolytes is essential in patients with risk factors predisposing to QTc prolongation.

  View details for DOI 10.1016/j.jpeds.2019.10.018

  View details for PubMedID 31761428

• Cardiac resynchronization and implantable defibrillators in adults with congenital heart disease. Heart failure reviews Chubb, H., Motonaga, K. S. 2019

  Abstract

  Cardiac resynchronization therapy (CRT) and implantable cardioverter defibrillators (ICDs) are well-established therapies for adult patients with heart failure that have been shown to improve morbidity and mortality. However, the benefits and indications for use in adults with congenital heart disease (ACHD) are less defined with no significant large prospective studies in this population. There are, however, multiple retrospective studies that demonstrate the efficacy of these devices in the ACHD population. These indicate a role for both CRT and ICDs in select patients with ACHD. The clinician and patient must balance the risks and benefits, summarized in complex evidence that reflects the heterogeneity of the ACHD patient group, and apply them in a patient-specific manner to optimize the utility of CRT and ICDs.

  View details for DOI 10.1007/s10741-019-09886-y

  View details for PubMedID 31754907

• Broad Genetic Testing in a Clinical Setting Uncovers a High Prevalence of Titin Loss-of-Function Variants in Very Early-Onset Atrial Fibrillation. Circulation. Genomic and precision medicine Goodyer, W. R., Dunn, K., Caleshu, C., Jackson, M., Wylie, J., Moscarello, T., Platt, J., Reuter, C., Smith, A., Trela, A., Ceresnak, S. R., Motonaga, K. S., Ashley, E., Yang, P., Dubin, A. M., Perez, M. 2019

  Abstract

  Atrial fibrillation (AF) is the most common sustained arrhythmia, affecting approximately 34 million worldwide. The pathophysiology of AF remains incompletely understood but is clearly complex with multiple underlying genetic, physiologic and environmental factors. Very early-onset AF (vEAF) (defined here as onset <45 years and without significant comorbidities), while rare (only ~0.5-3% of AF cases), is highly heritable, with a greater prevalence of rare variants in genes previously associated with AF. Patients with vEAF, therefore, represent an ideal population for discovering novel genes involved in the underlying genetic basis of AF. Notably, the Framingham study showed that patients with AF without comorbidities have a three-fold higher risk for heart failure. Conversely, several forms of inherited cardiomyopathy have been strongly associated with AF suggestive of a shared etiology.

  View details for DOI 10.1161/CIRCGEN.119.002713

  View details for PubMedID 31638414

• Road to the Summit May Follow an Eccentric Path. Circulation. Arrhythmia and electrophysiology Motonaga, K. S., Hsia, H. H. 2019; 12 (8): e007691

  View details for DOI 10.1161/CIRCEP.119.007691

  View details for PubMedID 31401855

• Management of Asymptomatic Wolff-Parkinson-White Pattern by Pediatric Electrophysiologists. The Journal of pediatrics Chubb, H., Campbell, R. M., Motonaga, K. S., Ceresnak, S. R., Dubin, A. M. 2019

  Abstract

  OBJECTIVE: To determine the present-day approach of pediatric cardiac electrophysiologists to asymptomatic Wolff-Parkinson-White (WPW) pattern and to contrast to both published consensus statements and a similar survey.STUDY DESIGN: A questionnaire was sent to 266 Pediatric and Congenital Electrophysiology Society physician members in 25 countries; 21 questions from the 2003 survey were repeated, with new questions added regarding risk stratification and decision making.RESULTS: We received 113 responses from 13 countries, with responders having extensive electrophysiology experience (median 15years [IQR 8.5-25years]). Only 12 (11%) believed that intermittent pre-excitation and 37 (33%) that sudden loss of pre-excitation on exercise test were sufficient evidence of accessory pathway safety to avoid an invasive electrophysiology study. Optimal weight for electrophysiology study was 20kg (IQR 18-22.5kg), and 61% and 58% would then ablate all right-sided or left-sided accessory pathways, respectively, regardless of electrophysiological properties, whereas only 23% would ablate all septal accessory pathways (P<.001). Compared with 2003, respondents were more likely to consider inducible arrhythmia (77% vs 26%, P<.001) as sufficient indication alone for ablation.CONCLUSIONS: In the context of recent literature regarding the reliability of risk-stratification tools, most operators are now performing electrophysiology study for asymptomatic Wolff-Parkinson-White regardless of noninvasive findings. Many will then proceed to default ablation of all accessory pathways distant from critical conduction structures.

  View details for DOI 10.1016/j.jpeds.2019.05.058

  View details for PubMedID 31235382

• A randomized placebo-controlled pilot trial shows that intranasal vasopressin improves social deficits in children with autism SCIENCE TRANSLATIONAL MEDICINE Parker, K. J., Oztan, O., Libove, R. A., Mohsin, N., Karhson, D. S., Sumiyoshi, R. D., Summers, J. E., Hinman, K. E., Motonaga, K. S., Phillips, J. M., Carson, D. S., Fung, L. K., Garner, J. P., Hardan, A. Y. 2019; 11 (491)

  View details for DOI 10.1126/scitranslmed.aau7356

  View details for Web of Science ID 000467273900004

• A novel pacing maneuver to verify the postpacing interval minus the tachycardia cycle length while adjusting for decremental conduction: Using "dual-chamber entrainment" for improved supraventricular tachycardia discrimination HEART RHYTHM Kaiser, D. W., Nasir, J. M., Liem, L., Brodt, C., Motonaga, K. S., Ceresnak, S. R., Turakhia, M. P., Dubin, A. M. 2019; 16 (5): 717–23

  View details for DOI 10.1016/j.hrthm.2018.11.021

  View details for Web of Science ID 000466096200013

• Extended cardiac ambulatory rhythm monitoring in adults with congenital heart disease: Arrhythmia detection and impact of extended monitoring CONGENITAL HEART DISEASE Schultz, K. E., Lui, G. K., McElhinney, D. B., Long, J., Balasubramanian, V., Sakarovitch, C., Fernandes, S. M., Dubin, A. M., Rogers, I. S., Romfh, A. W., Motonaga, K. S., Viswanathan, M. N., Ceresnak, S. R. 2019; 14 (3): 410–18

  View details for DOI 10.1111/chd.12736

  View details for Web of Science ID 000471070900013

• Extended cardiac ambulatory rhythm monitoring in adults with congenital heart disease: Arrhythmia detection and impact of extended monitoring. Congenital heart disease Schultz, K. E., Lui, G. K., McElhinney, D. B., Long, J., Balasubramanian, V., Sakarovitch, C., Fernandes, S. M., Dubin, A. M., Rogers, I. S., Romfh, A. W., Motonaga, K. S., Viswanathan, M. N., Ceresnak, S. R. 2019

  Abstract

  BACKGROUND: Arrhythmias are a leading cause of death in adults with congenital heart disease (ACHD). While 24-48-hour monitors are often used to assess arrhythmia burden, extended continuous ambulatory rhythm monitors (ECAM) can record 2 weeks of data. The utility of this device and the arrhythmia burden identified beyond 48-hour monitoring have not been evaluated in the ACHD population. Additionally, the impact of ECAM has not been studied to determine management recommendations.OBJECTIVE: To address the preliminary question, we hypothesized that clinically significant arrhythmias would be detected on ECAM beyond 48hours and this would lead to clinical management changes.METHODS: A single center retrospective cohort study of ACHD patients undergoing ECAM from June 2013 to May 2016 was performed. The number and type of arrhythmias detected within and beyond the first 48hours of monitoring were compared using Kaplan-Meier curves and Cox proportional hazard models.RESULTS: Three hundred fourteen patients had monitors performed [median age 31 (IQR 25-41) years, 61% female). Significant arrhythmias were identified in 156 patients (50%), of which 46% were noted within 48 hours. A management change based on an arrhythmia was made in 49 patients (16%).CONCLUSIONS: ECAM detects more clinically significant arrhythmias than standard 48-hour monitoring in ACHD patients. Management changes, including medication changes, further testing or imaging, and procedures, were made based on results of ECAM. Recommendations and guidelines have been made based on arrhythmias on 48-hour monitoring; the predictive ability and clinical consequence of arrhythmias found on ECAM are not yet known.

  View details for PubMedID 30604934

• A randomized placebo-controlled pilot trial shows that intranasal vasopressin improves social deficits in children with autism. Science translational medicine Parker, K. J., Oztan, O. n., Libove, R. A., Mohsin, N. n., Karhson, D. S., Sumiyoshi, R. D., Summers, J. E., Hinman, K. E., Motonaga, K. S., Phillips, J. M., Carson, D. S., Fung, L. K., Garner, J. P., Hardan, A. Y. 2019

  Abstract

  The social impairments of autism spectrum disorder (ASD) have a major impact on quality of life, yet there are no medications that effectively treat these core social behavior deficits. Preclinical research suggests that arginine vasopressin (AVP), a neuropeptide involved in promoting mammalian social behaviors, may be a possible treatment for ASD. Using a double-blind, randomized, placebo-controlled, parallel study design, we tested the efficacy and tolerability of a 4-week intranasal AVP daily treatment in 30 children with ASD. AVP-treated participants aged 6 to 9.5 years received the maximum daily target dose of 24 International Units (IU); participants aged 9.6 to 12.9 years received the maximum daily target dose of 32 IU. Intranasal AVP treatment compared to placebo enhanced social abilities as assessed by change from baseline in this phase 2 trial's primary outcome measure, the Social Responsiveness Scale, 2nd Edition total score (SRS-2 T score; F1,20 = 9.853; P = 0.0052; ηp2 = 33.0%; Cohen's d = 1.40). AVP treatment also diminished anxiety symptoms and some repetitive behaviors. Most of these findings were more pronounced when we accounted for pretreatment AVP concentrations in blood. AVP was well tolerated with minimal side effects. No AVP-treated participants dropped out of the trial, and there were no differences in the rate of adverse events reported between treatment conditions. Last, no changes from baseline were observed in vital signs, electrocardiogram tracings, height and body weight, or clinical chemistry measurements after 4 weeks of AVP treatment. These preliminary findings suggest that AVP has potential for treating social impairments in children with ASD.

  View details for PubMedID 31043522

• A Prospective Assessment of Optimal Mechanical Ventilation Parameters for Pediatric Catheter Ablation PEDIATRIC CARDIOLOGY Janson, C. M., Ceresnak, S. R., Choi, J. M., Dubin, A. M., Motonaga, K. S., Mann, G. E., Kahana, M., Fitz-James, I., Wise-Faberowski, L., Kamra, K., Nappo, L., Trela, A., Pass, R. H. 2019; 40 (1): 126–32

  Abstract

  Catheter stability, an important factor in ablation success, is affected by ventilation. Optimal ventilation strategies for pediatric catheter ablation are not known. We hypothesized that small tidal volume and positive end-expiratory pressure are associated with reduced ablation catheter movement at annular positions. Subjects aged 5-25 years undergoing ablation for supraventricular tachycardia (SVT) or WPW at two centers from March 2015 to September 2016 were prospectively enrolled and randomized to receive mechanical ventilation with either positive end-expiratory pressure of 5 cm H2O (PEEP) or 0 cm H2O (ZEEP). Movement of the ablation catheter tip at standard annular positions was measured using 3D electroanatomic mapping systems under two conditions: small tidal volume (STV) (3-5 mL/kg) or large TV (LTV) (6-8 mL/kg). 58 subjects (mean age 13.8 years) were enrolled for a total of 266 separate observations of catheter movement. STV ventilation was associated with significantly reduced catheter movement, compared to LTV at all positions (right posteroseptal: 2.5 ± 1.4 vs. 5.2 ± 3.1 mm, p < 0.0001; right lateral: 2.7 ± 1.6 vs. 6.3 ± 3.5 mm, p < 0.0001; left lateral: 1.8 ± 1.0 vs. 4.3 ± 1.9 mm, p < 0.0001). The presence or absence of PEEP had no effect on catheter movement. In multivariable analysis, STV was associated with a 3.1-mm reduction in movement (95% CI 2.6-3.5, p < 0.0001), adjusting for end-expiratory pressure, annular location, and patient size. We conclude that STV ventilation is associated with reduced ablation catheter movement compared to a LTV strategy, independent of PEEP and annular position.

  View details for PubMedID 30178187

• Validation of a novel automated signal analysis tool for ablation of Wolff-Parkinson-White Syndrome. PloS one Ceresnak, S. R., Pass, R. H., Dubin, A. M., Yang, L., Motonaga, K. S., Hedlin, H., Avasarala, K., Trela, A., McElhinney, D. B., Janson, C., Nappo, L., Ling, X. B., Gates, G. J. 2019; 14 (6): e0217282

  Abstract

  BACKGROUND: In previous pilot work we demonstrated that a novel automated signal analysis tool could accurately identify successful ablation sites during Wolff-Parkinson-White (WPW) ablation at a single center.OBJECTIVE: We sought to validate and refine this signal analysis tool in a larger multi-center cohort of children with WPW.METHODS: A retrospective review was performed of signal data from children with WPW who underwent ablation at two pediatric arrhythmia centers from 2008-2015. All patients with WPW ≤ 21 years who underwent invasive electrophysiology study and ablation with ablation signals available for review were included. Signals were excluded if temperature or power delivery was inadequate or lesion time was < 5 seconds. Ablation lesions were reviewed for each patient. Signals were classified as successful if there was loss of antegrade and retrograde accessory pathway (AP) conduction or unsuccessful if ablation did not eliminate AP conduction. Custom signal analysis software analyzed intracardiac electrograms for amplitudes, high and low frequency components, integrated area, and signal timing components to create a signal score. We validated the previously published signal score threshold 3.1 in this larger, more diverse cohort and explored additional scoring options. Logistic regression with lasso regularization using Youden's index criterion and a cost-benefit criterion to identify thresholds was considered as a refinement to this score.RESULTS: 347 signals (141 successful, 206 unsuccessful) in 144 pts were analyzed [mean age 13.2 ± 3.9 years, 96 (67%) male, 66 (45%) left sided APs]. The software correctly identified the signals as successful or unsuccessful in 276/347 (80%) at a threshold of 3.1. The performance of other thresholds did not significantly improve the predictive ability. A signal score threshold of 3.1 provided the following diagnostic accuracy for distinguishing a successful from unsuccessful signal: sensitivity 83%, specificity 77%, PPV 71%, NPV 87%.CONCLUSIONS: An automated signal analysis software tool reliably distinguished successful versus unsuccessful ablation electrograms in children with WPW when validated in a large, diverse cohort. Refining the tools using an alternative threshold and statistical method did not improve the original signal score at a threshold of 3.1. This software was effective across two centers and multiple operators and may be an effective tool for ablation of WPW.

  View details for DOI 10.1371/journal.pone.0217282

  View details for PubMedID 31242221

• A proposed method for the calculation of age-dependent QRS duration z-scores. Journal of electrocardiology Chubb, H. n., Ceresnak, S. R., Motonaga, K. S., Dubin, A. M. 2019; 58: 132–34

  Abstract

  There are currently no published algorithms for calculation of age-dependent QRS duration z-scores. The absence of a standardized measure has limited researchers' abilities to compare ECG measurements of electrical synchrony between subjects of different ages or longitudinally over time.Four existing studies of normal ECG measurements (total 19,062 subjects) were used to estimate age and sex-dependent means and standard deviations.Weighted means and standard deviations were best estimated by cubic functions to create z-score algorithms.Nomograms and algorithms for QRS duration z-scores may be estimated to compare ECG findings in both children and adults.

  View details for DOI 10.1016/j.jelectrocard.2019.12.004

  View details for PubMedID 31846856

• A Novel Pacing Maneuver to Verify the Post-Pacing Interval Minus the Tachycardia Cycle Length While Adjusting for Decremental Conduction: Using 'Dual Chamber Entrainment' for Improved Supraventricular Tachycardia Discrimination. Heart rhythm Kaiser, D. W., Nasir, J. M., Liem, L. B., Brodt, C., Motonaga, K. S., Ceresnak, S. R., Turakhia, M. P., Dubin, A. M. 2018

  Abstract

  BACKGROUND: The post-pacing interval (PPI) minus the tachycardia cycle length (TCL) is frequently used to investigate tachycardias. However, a variety of issues (e.g. failure to entrain, decremental conduction, and oscillating TCLs) can make interpretation of the PPI-TCL challenging.OBJECTIVES: To investigate a novel maneuver to confirm the PPI-TCL value without using either the ventricular PPI or the TCL interval. 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 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: [PPIV-TCL=Stim(AoV)+Stim(VoA)-PPIA].RESULTS: The PPI-TCL calculated by dual chamber entrainment highly correlated with the observed and AH-corrected PPI-TCL [R2=0.79 and 0.96, respectively, p<0.001]. A dual chamber entrainment PPI-TCL value of 80ms 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±10ms of additional decremental conduction beyond AH-prolongation, including four pathways with significant (>10ms) 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 >80ms favors AVNRT over ORT. This maneuver can be used to verify the observed PPI-TCL value in challenging cases.

  View details for PubMedID 30465902

• Antibiotic Prophylaxis Practices in Pediatric Cardiac Implantable Electronic Device Procedures: A Survey of the Pediatric And Congenital Electrophysiology Society (PACES) PEDIATRIC CARDIOLOGY Chen, S. Y., Ceresnak, S. R., Motonaga, K. S., Trela, A., Hanisch, D., Dubin, A. M. 2018; 39 (6): 1129–33

  View details for DOI 10.1007/s00246-018-1865-0

  View details for Web of Science ID 000441721000009

• Life-Threatening Event Risk in Children With Wolff-Parkinson-White Syndrome: A Multicenter International Study. JACC. Clinical electrophysiology Etheridge, S. P., Escudero, C. A., Blaufox, A. D., Law, I. H., Dechert-Crooks, B. E., Stephenson, E. A., Dubin, A. M., Ceresnak, S. R., Motonaga, K. S., Skinner, J. R., Marcondes, L. D., Perry, J. C., Collins, K. K., Seslar, S. P., Cabrera, M., Uzun, O., Cannon, B. C., Aziz, P. F., Kubus, P., Tanel, R. E., Valdes, S. O., Sami, S., Kertesz, N. J., Maldonado, J., Erickson, C., Moore, J. P., Asakai, H., Mill, L., Abcede, M., Spector, Z. Z., Menon, S., Shwayder, M., Bradley, D. J., Cohen, M. I., Sanatani, S. 2018; 4 (4): 433–44

  Abstract

  OBJECTIVES: This study sought to characterize risk in children with Wolff-Parkinson-White (WPW) syndrome by comparing those who had experienced a life-threatening event (LTE) with a control population.BACKGROUND: Children with WPW syndrome are at risk of sudden death.METHODS: This retrospective multicenter pediatric study identified 912 subjects≤21 years of age with WPW syndrome, using electrophysiology (EPS) studies. Case subjects had a history of LTE: sudden death, aborted sudden death, or atrialfibrillation (shortest pre-excited RR interval in atrial fibrillation [SPERRI] of≤250 ms or with hemodynamic compromise); whereas subjects did not. We compared clinical and EPS data between cases and subjects.RESULTS: Case subjects (n= 96) were older and less likely than subjects (n= 816) to have symptoms or documented tachycardia. Mean age at LTE was 14.1 ± 3.9 years of age. The LTE was the sentinel symptom in 65%, consisting of rapidly conducted pre-excited atrial fibrillation (49%), aborted sudden death (45%), and sudden death (6%). Three risk components were considered at EPS: SPERRI, accessory pathway effective refractory period (APERP), and shortest paced cycle length with pre-excitation during atrial pacing (SPPCL), and all were shorter in cases than in control subjects. In multivariate analysis, risk factors for LTE included male sex, Ebstein malformation, rapid anterograde conduction (APERP, SPERRI, or SPPCL≤250 ms), multiple pathways, and inducible atrial fibrillation. Of case subjects, 60 of 86 (69%) had≥2 EPS risk stratification components performed; 22 of 60 (37%) did not have EPS-determined high-risk characteristics, and 15 of 60 (25%) had neither concerning pathway characteristics nor inducible atrioventricular reciprocating tachycardia.CONCLUSIONS: Young patients may experience LTE from WPW syndrome without prior symptoms or markers of high-risk on EPS.

  View details for PubMedID 30067481

• Life-Threatening Event Risk in Children With Wolff-Parkinson-White Syndrome A Multicenter International Study JACC-CLINICAL ELECTROPHYSIOLOGY Etheridge, S. P., Escudero, C. A., Blaufox, A. D., Law, I. H., Dechert-Crooks, B. E., Stephenson, E. A., Dubin, A. M., Ceresnak, S. R., Motonaga, K. S., Skinner, J. R., Marcondes, L. D., Perry, J. C., Collins, K. K., Seslar, S. P., Cabrera, M., Uzun, O., Cannon, B. C., Aziz, P. F., Kubu, P., Tanel, R. E., Valdes, S. O., Sami, S., Kertesz, N. J., Maldonado, J., Erickson, C., Moore, J. P., Asakai, H., Mill, L., Abcede, M., Spector, Z. Z., Menon, S., Shwayder, M., Bradley, D. J., Cohen, M., Sanatani, S. 2018; 4 (4): 433–44

  View details for DOI 10.1016/j.jacep.2017.10.009

  View details for Web of Science ID 000602285700002

• Antibiotic Prophylaxis Practices in Pediatric Cardiac Implantable Electronic Device Procedures: A Survey of the Pediatric And Congenital Electrophysiology Society (PACES). Pediatric cardiology Chen, S. Y., Ceresnak, S. R., Motonaga, K. S., Trela, A., Hanisch, D., Dubin, A. M. 2018

  Abstract

  Cardiac implantable electronic device (CIED) infections are associated with significant morbidity in the pediatric device population, with a tenfold higher risk of infection in children compared to adults. The 2010 American Heart Association (AHA) guidelines recommend a single dose of systemic antibiotic (ABX) prophylaxis prior to CIED implantation and no post-operative (OP) ABX. However, there is limited data regarding adherence to this recommendation among the pediatric community. To assess current clinical practices for CIED ABX prophylaxis in pediatrics; whether the AHA guidelines are being followed; and if not, the reasons for non-adherence. An anonymous web-based survey was sent to physician members of the Pediatric And Congenital Electrophysiology Society regarding ABX prophylaxis for new CIED implants and reoperations. 75 (25%) members responded. Only 7% of respondents follow the 2010 AHA guidelines. While all respondents give pre-OP IV ABX, 64% routinely treat patients with 24-h post-OP IV ABX with additional oral or IV therapy. 69% of respondents are cognizant of the guidelines but 88% of those cognizant do not follow the guidelines for a variety of reasons including lack of data and different substrate (pediatric patients). 79% stated that pediatric-specific data would be required for them to change their practice and follow the published guidelines. The majority of pediatric EP physicians who responded to this survey do not follow the current AHA guidelines on ABX prophylaxis and administer post-OP ABX. Most pediatric EP physicians believe that the increased risk of infection in children merits additional ABX.

  View details for PubMedID 29564522

• THE DEVELOPMENT AND EFFICACY OF A PEDIATRIC CARDIOLOGY FELLOWSHIP ONLINE PREPARATORY COURSE Motonaga, K., Sacks, L., Olson, I., Balasubramanian, S., Chen, S., Peng, L., Feinstein, J., Silverman, N., Hanley, F., Axelrod, D., Krawczeski, C., Ceresnak, S. ELSEVIER SCIENCE INC. 2018: 2622

  View details for DOI 10.1016/S0735-1097(18)33163-2

  View details for Web of Science ID 000429659705072

• Bridge to success: A better method of cryoablation for atrioventricular nodal reentrant tachycardia in children HEART RHYTHM Reddy, C. D., Ceresnak, S. R., Motonaga, K. S., Avasarala, K., Feller, C., Trela, A., Hanisch, D., Dubin, A. M. 2017; 14 (11): 1649–54

  Abstract

  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.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.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.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.Voltage gradient mapping and longer lesion time can decrease recurrence rates in pediatric patients with AVNRT.

  View details for PubMedID 28716699

• Intranasal oxytocin treatment for social deficits and biomarkers of response in children with autism. Proceedings of the National Academy of Sciences of the United States of America Parker, K. J., Oztan, O., Libove, R. A., Sumiyoshi, R. D., Jackson, L. P., Karhson, D. S., Summers, J. E., Hinman, K. E., Motonaga, K. S., Phillips, J. M., Carson, D. S., Garner, J. P., Hardan, A. Y. 2017; 114 (30): 8119-8124

  Abstract

  Autism spectrum disorder (ASD) is characterized by core social deficits. Prognosis is poor, in part, because existing medications target only associated ASD features. Emerging evidence suggests that the neuropeptide oxytocin (OXT) may be a blood-based biomarker of social functioning and a possible treatment for ASD. However, prior OXT treatment trials have produced equivocal results, perhaps because of variability in patients' underlying neuropeptide biology, but this hypothesis has not been tested. Using a double-blind, randomized, placebo-controlled, parallel design, we tested the efficacy and tolerability of 4-wk intranasal OXT treatment (24 International Units, twice daily) in 32 children with ASD, aged 6-12 y. When pretreatment neuropeptide measures were included in the statistical model, OXT compared with placebo treatment significantly enhanced social abilities in children with ASD [as measured by the trial's primary outcome measure, the Social Responsiveness Scale (SRS)]. Importantly, pretreatment blood OXT concentrations also predicted treatment response, such that individuals with the lowest pretreatment OXT concentrations showed the greatest social improvement. OXT was well tolerated, and its effects were specific to social functioning, with no observed decrease in repetitive behaviors or anxiety. Finally, as with many trials, some placebo-treated participants showed improvement on the SRS. This enhanced social functioning was mirrored by a posttreatment increase in their blood OXT concentrations, suggesting that increased endogenous OXT secretion may underlie this improvement. These findings indicate that OXT treatment enhances social abilities in children with ASD and that individuals with pretreatment OXT signaling deficits may stand to benefit the most from OXT treatment.

  View details for DOI 10.1073/pnas.1705521114

  View details for PubMedID 28696286

  View details for PubMedCentralID PMC5544319

• A multicenter review of ablation in the aortic cusps in young people. Pacing and clinical electrophysiology : PACE Nguyen, M. B., Ceresnak, S. R., Janson, C. M., Fishberger, S. B., Love, B. A., Blaufox, A. D., Motonaga, K. S., Dubin, A. M., Nappo, L., Pass, R. H. 2017

  Abstract

  Ablation within the aortic cusp is safe and effective in adults. There are little data on aortic cusp ablation in the pediatric literature. We investigated the safety and efficacy of aortic cusp ablation in young patients.A retrospective, descriptive study of aortic cusp ablation in five pediatric electrophysiology centers from 2008 to 2014 was performed. All patients <21 years of age who underwent ablation in the aortic cusps were included. Factors analyzed included patient demographics, procedural details, outcomes, and complications.Thirteen patients met inclusion criteria (median age 16 years [range 10-20.5] and median body surface area 1.58 m(2) [range 1.12-2.33]). Substrates for ablation included: nine premature ventricular contractions or sustained ventricular tachycardia (69%), two concealed anteroseptal accessory pathways (APs) (15%), one Wolff-Parkinson-White with an anteroseptal AP (8%), and one ectopic atrial tachycardia (8%). Three-dimensional electroanatomic mapping in combination with fluoroscopy was used in 12/13 (92%) patients. Standard 4-mm-tip radiofrequency (RF) current was used in 11/13 (85%) and low-power irrigated-tip RF in 2/13 (15%). Angiography was used in 13/13 and intracardiac echocardiography was additionally utilized in 3/13 (23%). Ablation locations included: eight noncoronary (62%), three left (23%), and two right (15%) cusps. Ablation was acutely successful in all patients. At median follow-up of 20 months, there was one recurrence of PVCs (8%). There were no ablation-related complications and no valvular injuries observed.Arrhythmias originating from the coronary cusps in this series were successfully and safely ablated in young people without injury to the coronary arteries or the aortic valve.

  View details for DOI 10.1111/pace.13126

  View details for PubMedID 28568013

• Ventricular pacing in single ventricles-A bad combination. Heart rhythm Bulic, A., Zimmerman, F. J., Ceresnak, S. R., Shetty, I., Motonaga, K. S., Freter, A., Trela, A. V., Hanisch, D., Russo, L., Avasarala, K., Dubin, A. M. 2017; 14 (6): 853-857

  Abstract

  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.To determine the longitudinal effect of VP in SV patients.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.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).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.

  View details for DOI 10.1016/j.hrthm.2017.03.035

  View details for PubMedID 28528723

• Advances in Pediatric Cardiology Boot Camp: Boot Camp Training Promotes Fellowship Readiness and Enables Retention of Knowledge. Pediatric cardiology Ceresnak, S. R., Axelrod, D. M., Sacks, L. D., Motonaga, K. S., Johnson, E. R., Krawczeski, C. D. 2017

  Abstract

  We previously demonstrated that a pediatric cardiology boot camp can improve knowledge acquisition and decrease anxiety for trainees. We sought to determine if boot camp participants entered fellowship with a knowledge advantage over fellows who did not attend and if there was moderate-term retention of that knowledge. A 2-day training program was provided for incoming pediatric cardiology fellows from eight fellowship programs in April 2016. Hands-on, immersive experiences and simulations were provided in all major areas of pediatric cardiology. Knowledge-based examinations were completed by each participant prior to boot camp (PRE), immediately post-training (POST), and prior to the start of fellowship in June 2016 (F/U). A control group of fellows who did not attend boot camp also completed an examination prior to fellowship (CTRL). Comparisons of scores were made for individual participants and between participants and controls. A total of 16 participants and 16 control subjects were included. Baseline exam scores were similar between participants and controls (PRE 47 ± 11% vs. CTRL 52 ± 10%; p = 0.22). Participants' knowledge improved with boot camp training (PRE 47 ± 11% vs. POST 70 ± 8%; p < 0.001) and there was excellent moderate-term retention of the information taught at boot camp (PRE 47 ± 11% vs. F/U 71 ± 8%; p < 0.001). Testing done at the beginning of fellowship demonstrated significantly better scores in participants versus controls (F/U 71 ± 8% vs. CTRL 52 ± 10%; p < 0.001). Boot camp participants demonstrated a significant improvement in basic cardiology knowledge after the training program and had excellent moderate-term retention of that knowledge. Participants began fellowship with a larger fund of knowledge than those fellows who did not attend.

  View details for DOI 10.1007/s00246-016-1560-y

  View details for PubMedID 28161811

• Intranasal oxytocin treatment for social deficits and biomarkers of response in children with autism. Proceedings of the National Academy of Sciences Parker, K. J., Oztan, O., Libove, R. A., Sumiyoshi, R. D., Jackson, L. P., Karhson, D. S., Summers, J. E., Hinman, K. E., Motonaga, K. S., Phillips, J. M., Carson, D. S., Garner, J. P., Hardan, A. Y. 2017; 114 (30): 8119-8124

  Abstract

  Autism spectrum disorder (ASD) is characterized by core social deficits. Prognosis is poor, in part, because existing medications target only associated ASD features. Emerging evidence suggests that the neuropeptide oxytocin (OXT) may be a blood-based biomarker of social functioning and a possible treatment for ASD. However, prior OXT treatment trials have produced equivocal results, perhaps because of variability in patients' underlying neuropeptide biology, but this hypothesis has not been tested. Using a double-blind, randomized, placebo-controlled, parallel design, we tested the efficacy and tolerability of 4-wk intranasal OXT treatment (24 International Units, twice daily) in 32 children with ASD, aged 6-12 y. When pretreatment neuropeptide measures were included in the statistical model, OXT compared with placebo treatment significantly enhanced social abilities in children with ASD [as measured by the trial's primary outcome measure, the Social Responsiveness Scale (SRS)]. Importantly, pretreatment blood OXT concentrations also predicted treatment response, such that individuals with the lowest pretreatment OXT concentrations showed the greatest social improvement. OXT was well tolerated, and its effects were specific to social functioning, with no observed decrease in repetitive behaviors or anxiety. Finally, as with many trials, some placebo-treated participants showed improvement on the SRS. This enhanced social functioning was mirrored by a posttreatment increase in their blood OXT concentrations, suggesting that increased endogenous OXT secretion may underlie this improvement. These findings indicate that OXT treatment enhances social abilities in children with ASD and that individuals with pretreatment OXT signaling deficits may stand to benefit the most from OXT treatment.

  View details for DOI 10.1073/pnas.1705521114

  View details for PubMedCentralID PMC5544319

• Tachycardia cycle and atrioventricular nodal conduction properties in children with supraventricular tachycardia. Pacing and clinical electrophysiology : PACE Mills, M., Dubin, A. M., Motonaga, K. S., Ceresnak, S. R. 2017; 40 (6): 745–47

  View details for PubMedID 28383202

• Intranasal Vasopressin Treatment Improves Social Abilities in Children With Autism Parker, K., Oztan, O., Libove, R., Sumiyoshi, R., Summers, J., Hinman, K., Fung, L., Motonaga, K., Carson, D., Phillips, J., Garner, J., Hardan, A. NATURE PUBLISHING GROUP. 2016: S341

  View details for Web of Science ID 000440365600575

• Is There a Difference in Tachycardia Cycle Length during SVT in Children with AVRT and AVNRT? Pacing and clinical electrophysiology : PACE Mills, M. F., Motonaga, K. S., Trela, A., Dubin, A. M., Avasarala, K., Ceresnak, S. R. 2016

  Abstract

  There are limited adult data suggesting the tachycardia cycle length (TCL) of atrioventricular reentry tachycardia (AVRT) is shorter than atrioventricular nodal reentry tachycardia (AVNRT), though little data exist in children. We sought to determine if there is a difference in TCL between AVRT and AVNRT in children.A single-center retrospective review of children with supraventricular tachycardia (SVT) from 2000 to 2015 was performed.Age ≤ 18 years, invasive electrophysiology study (EPS) confirming AVRT or AVNRT.Atypical AVNRT, congenital heart disease, antiarrhythmic medication use at time of EPS. Data were compared between patients with AVRT and AVNRT via t-test, χ(2) test, and linear regression.A total of 835 patients were included (12 ± 4 years, 52 ± 31 kg, TCL 321 ± 55 ms), 539 (65%) with AVRT (270 Wolff-Parkinson-White, 269 concealed pathways) and 296 (35%) with AVNRT. Patients with AVRT were younger (11.7 ± 4.1 years vs 13.0 ± 3.6 years, P < 0.001) and smaller (49 ± 22 kg vs 57 ± 43 kg, P < 0.001). In the baseline state, the TCL was shorter in AVRT than AVRNT (329 ± 51 ms vs 340 ± 60 ms, P = 0.04). In patients requiring isoproterenol to induce SVT, there was no difference in TCL (290 ± 49 ms vs 297 ± 49 ms, P = 0.26). When controlling for age, there was no difference in TCL between AVRT and AVNRT at baseline or on isoproterenol. The regression equation for TCL in the baseline state was TCL = 290 + 4 (age), indicating the TCL will increase by 4 ms above a baseline of 290 ms for each year of life.When controlling for age, there is no difference in the TCL between AVRT and AVNRT in children. Age, not tachycardia mechanism, is the most significant factor in predicting TCL.

  View details for DOI 10.1111/pace.12950

  View details for PubMedID 27653639

• Risk of cardiac disease and observations on lack of potential predictors by clinical history among children presenting for cardiac evaluation of mid-exertional syncope CARDIOLOGY IN THE YOUNG Miyake, C. Y., Motonaga, K. S., Fischer-Colbrie, M. E., Chen, L., Hanisch, D. G., Balise, R. R., Kim, J. J., Dubin, A. M. 2016; 26 (5): 894-900

  Abstract

  This study aimed to evaluate the incidence of cardiac disorders among children with mid-exertional syncope evaluated by a paediatric cardiologist, determine how often a diagnosis was not established, and define potential predictors to differentiate cardiac from non-cardiac causes. Study design We carried out a single-centre, retrospective review of children who presented for cardiac evaluation due to a history of exertional syncope between 1999 and 2012. Inclusion criteria included the following: (1) age ⩽18 years; (2) mid-exertional syncope; (3) electrocardiogram, echocardiogram and an exercise stress test, electrophysiology study, or tilt test, with exception of long QT, which did not require additional testing; and (4) evaluation by a paediatric cardiologist. Mid-exertional syncope was defined as loss of consciousness in the midst of active physical activity. Patients with peri-exertional syncope immediately surrounding but not during active physical exertion were excluded.A total of 60 patients met the criteria for mid-exertional syncope; 32 (53%) were diagnosed with cardiac syncope and 28 with non-cardiac syncope. A majority of cardiac patients were diagnosed with an electrical myopathy, the most common being Long QT syndrome. In nearly half of the patients, a diagnosis could not be established or syncope was felt to be vasovagal in nature. Neither the type of exertional activity nor the symptoms or lack of symptoms occurring before, immediately preceding, and after the syncopal event differentiated those with or without a cardiac diagnosis.Children with mid-exertional syncope are at risk for cardiac disease and warrant evaluation. Reported symptoms may not differentiate benign causes from life-threatening disease.

  View details for DOI 10.1017/S1047951115001481

  View details for PubMedID 26277987

• Electrocardiographic repolarization abnormalities and increased risk of life-threatening arrhythmias in children with dilated cardiomyopathy HEART RHYTHM Chen, S., Motonaga, K. S., Hollander, S. A., Almond, C. S., Rosenthal, D. N., Kaufman, B. D., May, L. J., Avasarala, K., Dao, D. T., Dubin, A. M., Ceresnak, S. R. 2016; 13 (6): 1289-1296

  Abstract

  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.We sought to determine the associations between ECG repolarization abnormalities and LTEs in children with DCM.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.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.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.

  View details for DOI 10.1016/j.hrthm.2016.02.014

  View details for Web of Science ID 000376334800016

  View details for PubMedID 26945851

• Pediatric Cardiology Boot Camp: Description and Evaluation of a Novel Intensive Training Program for Pediatric Cardiology Trainees PEDIATRIC CARDIOLOGY Ceresnak, S. R., Axelrod, D. M., Motonaga, K. S., Johnson, E. R., Krawczeski, C. D. 2016; 37 (5): 834-844

  Abstract

  The transition from residency to subspecialty fellowship in a procedurally driven field such as pediatric cardiology is challenging for trainees. We describe and assess the educational value of a pediatric cardiology "boot camp" educational tool designed to help prepare trainees for cardiology fellowship. A two-day intensive training program was provided for pediatric cardiology fellows in July 2015 at a large fellowship training program. Hands-on experiences and simulations were provided in: anatomy, auscultation, echocardiography, catheterization, cardiovascular intensive care (CVICU), electrophysiology (EP), heart failure, and cardiac surgery. Knowledge-based exams as well as surveys were completed by each participant pre-training and post-training. Pre- and post-exam results were compared via paired t tests, and survey results were compared via Wilcoxon rank sum. A total of eight participants were included. After boot camp, there was a significant improvement between pre- and post-exam scores (PRE 54 ± 9 % vs. POST 85 ± 8 %; p ≤ 0.001). On pre-training survey, the most common concerns about starting fellowship included: CVICU emergencies, technical aspects of the catheterization/EP labs, using temporary and permanent pacemakers/implantable cardiac defibrillators (ICDs), and ECG interpretation. Comparing pre- and post-surveys, there was a statistically significant improvement in the participants comfort level in 33 of 36 (92 %) areas of assessment. All participants (8/8, 100 %) strongly agreed that the boot camp was a valuable learning experience and helped to alleviate anxieties about the start of fellowship. A pediatric cardiology boot camp experience at the start of cardiology fellowship can provide a strong foundation and serve as an educational springboard for pediatric cardiology fellows.

  View details for DOI 10.1007/s00246-016-1357-z

  View details for Web of Science ID 000377722400005

  View details for PubMedID 26961569

• IS THERE A DIFFERENCE IN TACHYCARDIA CYCLE LENGTH DURING SUPRAVENTRICULAR TACHYCARDIA IN CHILDREN WITH ATRIO-VENTRICULAR RECIPROCATING TACHYCARDIA AND ATRIO-VENTRICULAR NODAL RE-ENTRY TACHYCARDIA? Mills, M., Motonaga, K., Trela, A., Dubin, A., Avasarala, K., Ceresnak, S. ELSEVIER SCIENCE INC. 2016: 848

  View details for DOI 10.1016/S0735-1097(16)30849-X

  View details for Web of Science ID 000375188701692

• Unusual Outflow Tract Ventricular Tachycardia. Cardiac electrophysiology clinics Motonaga, K. S., Ceresnak, S. R., Hsia, H. H. 2016; 8 (1): 79-88

  Abstract

  Distinguishing premature ventricular contractions/ventricular tachycardia from the right ventricular outflow tract versus the left ventricular outflow tract can be difficult by electrocardiogram findings alone. A thorough understanding of the outflow tract anatomy and a systematic and meticulous approach to mapping of the ventricular outflow regions and great vessels increases the success rate and decreases the risk of damage to adjacent structures and the conduction system. The use of multimodality imaging, particularly real-time intracardiac echocardiographic guidance, is essential for defining anatomy, ensuring adequate catheter contact, and minimizing risks.

  View details for DOI 10.1016/j.ccep.2015.10.032

  View details for PubMedID 26920175

• A Pilot Study Assessing ECG versus ECHO Ventriculoventricular Optimization in Pediatric Resynchronization Patients. Journal of cardiovascular electrophysiology Punn, R., Hanisch, D., Motonaga, K. S., Rosenthal, D. N., Ceresnak, S. R., Dubin, A. M. 2016; 27 (2): 210-216

  Abstract

  Cardiac resynchronization therapy indications and management are well described in adults. Echocardiography (ECHO) has been used to optimize mechanical synchrony in these patients; however, there are issues with reproducibility and time intensity. Pediatric patients add challenges, with diverse substrates and limited capacity for cooperation. Electrocardiographic (ECG) methods to assess electrical synchrony are expeditious but have not been extensively studied in children. We sought to compare ECHO and ECG CRT optimization in children.Prospective, pediatric, single-center cross-over trial comparing ECHO and ECG optimization with CRT. Patients were assigned to undergo either ECHO or ECG optimization, followed for 6 months, and crossed-over to the other assignment for another 6 months. ECHO pulsed-wave tissue Doppler and 12-lead ECG were obtained for 5 VV delays. ECG optimization was defined as the shortest QRSD and ECHO optimization as the lowest dyssynchrony index. ECHOs/ECGs were interpreted by readers blinded to optimization technique. After each 6 month period, these data were collected: ejection fraction, velocimetry-derived cardiac index, quality of life, ECHO-derived stroke distance, M-mode dyssynchrony, study cost, and time. Outcomes for each optimization method were compared.From June 2012 to December 2013, 19 patients enrolled. Mean age was 9.1 ± 4.3 years; 14 (74%) had structural heart disease. The mean time for optimization was shorter using ECG than ECHO (9 ± 1 min vs. 68 ± 13 min, P < 0.01). Mean cost for charges was $4,400 ± 700 less for ECG. No other outcome differed between groups.ECHO optimization of synchrony was not superior to ECG optimization in this pilot study. ECG optimization required less time and cost than ECHO optimization.

  View details for DOI 10.1111/jce.12863

  View details for PubMedID 26515428

• Right-sided subcutaneous implantable cardioverter-defibrillator placement in a patient with dextrocardia, tetralogy of Fallot, and conduction disease. HeartRhythm case reports Ceresnak, S. R., Motonaga, K. S., Rogers, I. S., Viswanathan, M. N. 2015; 1 (4): 186-189

  View details for DOI 10.1016/j.hrcr.2015.02.001

  View details for PubMedID 28491545

• 50 is the new 70: Short ventriculoatrial times are common in children with atrioventricular reciprocating tachycardia. Heart rhythm Ceresnak, S. R., Doan, L. N., Motonaga, K. S., Avasarala, K., Trela, A. V., Reddy, C. D., Dubin, A. M. 2015; 12 (7): 1541-1547

  Abstract

  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.This study sought to determine the incidence and characteristics associated with short-VA AVRT in children.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.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).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.

  View details for DOI 10.1016/j.hrthm.2015.03.047

  View details for PubMedID 25828598

• Success Rates in Pediatric WPW Ablation Are Improved with 3-Dimensional Mapping Systems Compared with Fluoroscopy Alone: A Multicenter Study JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY Ceresnak, S. R., Dubin, A. M., Kim, J. J., Valdes, S. O., Fishberger, S. B., Shetty, I., Zimmerman, F., Tanel, R. E., Epstein, M. R., Motonaga, K. S., Capone, C. A., Nappo, L., Gates, G. J., Pass, R. H. 2015; 26 (4): 412-416

  Abstract

  Three-dimensional mapping (3-D) systems are frequently used for ablation of supraventricular tachycardia. Prior studies have demonstrated radiation dosage reduction with 3-D, but there are no data on whether 3-D improves the efficacy of ablation of Wolff-Parkinson-White syndrome (WPW). We sought to determine if 3-D improves the success rate for ablation of WPW in children.Multicenter retrospective study including patients ≤21 years of age with WPW undergoing ablation from 2008 to 2012. Success rates using the 2 techniques (3-D vs. fluoroscopy alone [FLUORO]) were compared.Six hundred and fifty-one cases were included (58% male, mean age 13 ± 4 years, 366 [56%] 3-D). Baseline characteristics including gender, weight, accessory pathway (AP) location, number of APs, and repeat ablation attempts were similar between the 2 groups (3-D and FLUORO) The 3-D group was slightly younger (12.7 ± 4.0 vs. 13.3 ± 4.0 years; P = 0.04) and less likely to undergo ablation utilizing cryoenergy (38 [10%] vs. 56 [20%]; P < 0.01). The 3-D group had a higher acute success rate of ablation (355 [97%] vs. 260 [91%]; P < 0.01). No differences were seen in recurrence (16 [5%] vs. 26 [9%]; P = 0.09) or complication rates (1 [0.3%] vs. 1 [0.4%]; P = 0.86) between the groups. On multivariable analysis, 3-D was shown to significantly improve success at ablation with an odds ratio of 3.1 (95% CI 1.44-6.72; P < 0.01).Use of 3-D significantly improved success rates for ablation of WPW in children. The increase in acute success associated with 3-D suggests it is an important adjunct for catheter ablation of WPW in children.

  View details for DOI 10.1111/jce.12623

  View details for Web of Science ID 000352814300012

  View details for PubMedID 25600208

• Diminished exercise capacity and chronotropic incompetence in pediatric patients with congenital complete heart block and chronic right ventricular pacing. Heart rhythm Motonaga, K. S., Punn, R., Axelrod, D. M., Ceresnak, S. R., Hanisch, D., Kazmucha, J. A., Dubin, A. M. 2015; 12 (3): 560-565

  Abstract

  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.The purpose of this study was to evaluate the exercise capacity and LV function in pediatric patients with CCAVB receiving chronic RV pacing.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.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).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.

  View details for DOI 10.1016/j.hrthm.2014.11.036

  View details for PubMedID 25433143

• Ventricular lead redundancy to prevent cardiovascular events and sudden death from lead fracture in pacemaker-dependent children. Heart rhythm Ceresnak, S. R., Perera, J. L., Motonaga, K. S., Avasarala, K., Malloy-Walton, L., Hanisch, D., Punn, R., Maeda, K., Reddy, V. M., Doan, L. N., Kirby, K., Dubin, A. M. 2015; 12 (1): 111-116

  Abstract

  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(CVE) and death.To determine if redundant ventricular lead systems(RVLS) can safeguard against CVE and death in PMD children.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 biventricular systems(BiV) systems placed for standard resynchronization indications were excluded. RVLS patients were compared to PMD patients with only a single pacing lead on the ventricle(SiV).769 patients underwent PM/ICD placement with 76 BiV implants and there were 49 PMD patients(6%). 13 patients underwent implantation of a 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 vs. SiV 9.4±6.7 years; p=0.52), weight(RVLS 38.2±32.6 vs. SiV 35.2±29.3 kg; p=0.62), 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.RVLS systems should be considered in children who are PMD and require permanent epicardial pacing. BiV pacing and RVLS may decrease the risk of CVEs in the event of lead failure in PMD patients.

  View details for DOI 10.1016/j.hrthm.2014.09.056

  View details for PubMedID 25277988

• Cardiac resynchronization therapy for pediatric patients with heart failure and congenital heart disease: a reappraisal of results. Circulation Motonaga, K. S., Dubin, A. M. 2014; 129 (18): 1879-1891

  View details for DOI 10.1161/CIRCULATIONAHA.113.001383

  View details for PubMedID 24799504

• Diagnosis and Management of Pediatric Brugada Syndrome: A Survey of Pediatric Electrophysiologists PACE-PACING AND CLINICAL ELECTROPHYSIOLOGY Harris, B. U., Miyake, C. Y., Motonaga, K. S., Dubin, A. M. 2014; 37 (5): 638-642

  Abstract

  Brugada syndrome (BrS) can be difficult to diagnose and treat, especially in the young patient. As there is currently no consensus on the evaluation and treatment of BrS in the pediatric population, we sought to describe the current practice for the diagnosis and treatment of BrS among pediatric electrophysiologists.A web-based survey was distributed to 204 physician members (MDs) of The Pediatric and Adult Congenital Electrophysiology Society (PACES). Practice characteristics, BrS patient attributes, and diagnostic and therapeutic preferences were collected.Responses were obtained from 83 pediatric electrophysiologists. The most common initial presentation was family history. There is a large variation in testing, particularly in the use of electrophysiology (EP) studies, drug challenge testing, and genetic testing. Despite limited treatment options, there is only consensus in the therapeutic approach to the pediatric patient with symptomatic BrS with 97% of physicians recommending an implantable cardioverter defibrillator (ICD). In the asymptomatic patient, a wide variation in therapy was seen with only 27% of physicians recommending an ICD CONCLUSIONS: Significant practice variation exists among pediatric electrophysiologists with deviation from accepted diagnostic and therapeutic practices for adult BrS patients. Further studies are necessary to establish best practice guidelines for BrS in the pediatric EP community.

  View details for DOI 10.1111/pace.12346

  View details for Web of Science ID 000334863000016

  View details for PubMedID 24456371

• In-hospital arrhythmia development and outcomes in pediatric patients with acute myocarditis. American journal of cardiology Miyake, C. Y., Teele, S. A., Chen, L., Motonaga, K. S., Dubin, A. M., Balasubramanian, S., Balise, R. R., Rosenthal, D. N., Alexander, M. E., Walsh, E. P., Mah, D. Y. 2014; 113 (3): 535-540

  Abstract

  Cardiac arrhythmias are a complication of myocarditis. There are no large studies of in-hospital arrhythmia development and outcomes in pediatric patients with acute myocarditis. This was a retrospective 2-center review of patients ≤21 years hospitalized with acute myocarditis from 1996 to 2012. Fulminant myocarditis was defined as the need for inotropic support within 24 hours of presentation. Acute arrhythmias occurred at presentation and subacute after admission. Eighty-five patients (59% men) presented at a median age of 10 years (1 day to 18 years). Arrhythmias occurred in 38 patients (45%): 16 acute, 12 subacute, and 9 acute and subacute (1 onset unknown). Arrhythmias were associated with low voltages on the electrocardiogram (14 of 34, 41% vs 6 of 47, 13%; odds ratio [OR] 4.78, 95% confidence interval [CI] 1.60 to 14.31) and worse outcome (mechanical support, orthotopic heart transplant, or death; OR 7.59, 95% CI 2.61 to 22.07) but were not statistically significantly associated with a fulminant course, ST changes, initial myocardial function, lactate, creatinine level, C-reactive protein and/or erythrocyte sedimentation rate, or troponin I level, after adjusting for multiple comparisons. Subacute arrhythmias were associated with preceding ST changes (10 of 15, 67% vs 15 of 59, 25%, OR 5.87, 95% CI 1.73 to 19.93). All patients surviving to discharge had arrhythmia resolution or control before discharge (10 on antiarrhythmic), with 1 exception (patient with complete heart block requiring a pacemaker). At 1-year follow-up, there were 3 recurrences of ventricular arrhythmias, but no arrhythmia-related mortality. In conclusion, arrhythmias are common in pediatric patients with myocarditis, occurring in nearly 1/2 of all hospitalized children and are associated with a worse outcome. Early identification of subacute arrhythmias using electrocardiographic changes may help management. A majority of patients do not require continued postdischarge arrhythmia treatment.

  View details for DOI 10.1016/j.amjcard.2013.10.021

  View details for PubMedID 24332245

• Electrophysiologic therapeutics in heart failure in adult congenital heart disease. Heart failure clinics Motonaga, K. S., Khairy, P., Dubin, A. M. 2014; 10 (1): 69-89

  Abstract

  Arrhythmias have long been recognized as a major cause of morbidity and mortality in the adult with congenital heart disease. It is important that the clinician accurately diagnoses these disturbances and is cognizant of the full array of antiarrhythmic agents and devices available to treat these conditions. This review discusses the most common arrhythmias encountered in this population and the therapeutic options available. Specific issues unique to this population are also addressed.

  View details for DOI 10.1016/j.hfc.2013.09.011

  View details for PubMedID 24275296

• Do pediatric electrophysiologists read pre-participation screening electrocardiograms more accurately than general pediatric cardiologists? journal of pediatrics Harbison, A. L., Hill, A. C., Motonaga, K. S., Miyake, C. Y., Dubin, A. M. 2013; 163 (6): 1775-1777

  Abstract

  Pre-participation electrocardiogram (ECG) screening of athletes is controversial. Pediatric electrophysiologists do not interpret screening ECGs more accurately than pediatric cardiologists with average number of correct ECG interpretations of 13.1-12.4 (P = .14). Electrophysiologists ordered fewer follow-up tests and were more likely to give sports recommendations based on published guidelines.

  View details for DOI 10.1016/j.jpeds.2013.07.034

  View details for PubMedID 23993128

• The effects of ketamine on dexmedetomidine-induced electrophysiologic changes in children. Paediatric anaesthesia Char, D., Drover, D. R., Motonaga, K. S., Gupta, S., Miyake, C. Y., Dubin, A. M., Hammer, G. B. 2013; 23 (10): 898-905

  Abstract

  BACKGROUND: Dexmedetomidine is an alpha2-adrenergic agonist used for sedation and analgesia in children. We previously showed that dexmedetomidine depresses sinus and AV nodal function resulting in adverse hemodynamic effects such as bradycardia and increased blood pressure. We hypothesized that these effects of dexmedetomidine might be antagonized by co-administration of ketamine, which has sympathomimetic properties. METHODS: Twenty-two children (ages 5-17 years) undergoing electrophysiologic (EP) study and ablation for supraventricular tachycardia were enrolled. Patients were kept sedated with continuous infusion of propofol at a fixed rate. Hemodynamic and EP parameters were measured before and after a loading dose of dexmedetomidine (1 μg·kg(-1) ). A continuous infusion of dexmedetomidine (0.7 μg·kg(-1) ·h(-1) ) was initiated and a ketamine loading dose (1 mg·kg(-1) ), followed by continuous infusion (1 mg·kg(-1) ·h(-1) ), was given. A repeat set of hemodynamic and EP parameters were then measured at the time of projected peak tissue concentration for both drugs. RESULTS: A significant increase in mean arterial pressure (MAP) was seen compared with baseline after loading of dexmedetomidine. This returned to baseline after co-administration of ketamine (mean difference between baseline and after ketamine 1.8 mmHg; 95%CI, -7.8 to 4.3; P = <0.001). A decrease in heart rate was seen after dexmedetomidine followed by a return to baseline after co-administration of ketamine (mean difference between baseline and after ketamine -6.5 bpm; 95%CI, -11.2 to -1.8; P = 0.005). Sinus node recovery time was lengthened after dexmedetomidine but returned to baseline after ketamine (mean difference between baseline and after ketamine -16.2 ms; 95%CI, -63 to 30; P = 0.014). QT was prolonged after dexmedetomidine and returned to baseline after ketamine (mean difference between baseline and after ketamine -34.2 ms; 95%CI, -48.4 to -20.2; P = 0.004). AV nodal effective refractory period was also impaired after dexmedetomidine and showed weak evidence for return to baseline function after ketamine (mean difference between baseline and after ketamine -22.8 ms; 95%CI, -40.2 to -5.2; P = 0.069). CONCLUSION: The concurrent use of ketamine may mitigate the negative chronotropic effects of dexmedetomidine.

  View details for DOI 10.1111/pan.12143

  View details for PubMedID 23506472

• Infant ventricular fibrillation after ST-segment changes and QRS widening: a new cause of sudden infant death? Circulation. Arrhythmia and electrophysiology Miyake, C. Y., Davis, A. M., Motonaga, K. S., Dubin, A. M., Berul, C. I., Cecchin, F. 2013; 6 (4): 712-718

  Abstract

  BACKGROUND: -Ventricular arrhythmia related sudden cardiac arrest in infants with structurally normal hearts is rare. There have been no previously published reports of infants less than 3 months of age with ventricular fibrillation in which a primary diagnosis could not be defined. METHODS AND RESULTS: -Retrospective chart review of 3 unrelated infants less than 2 months of age from 3 different tertiary care centers within the United States and Australia. All 3 infants survived sudden cardiac arrest secondary to multiple episodes of polymorphic ventricular tachycardia and ventricular fibrillation. Each infant demonstrated unique and transient ECG findings consisting of ST changes and QRS widening prior to arrhythmia onset that have not been previously reported. Amiodarone, sedation, sodium channel blocking agents and/or ventricular pacing were effective in suppressing acute events. Despite thorough investigation including genetic testing, the etiology of the ventricular arrhythmias in each of these infants remains unclear. CONCLUSIONS: -This is the first report of idiopathic ventricular fibrillation in young infants preceded by stereotypical transient ECG changes. These findings may represent a new, potentially treatable cause of sudden infant death. Recognition of these prodromal changes may be important in future management and survival of these infants.

  View details for DOI 10.1161/CIRCEP.113.000444

  View details for PubMedID 23748209

• Insights into dyssynchrony in Hypoplastic Left Heart Syndrome HEART RHYTHM Motonaga, K. S., Miyake, C. Y., Punn, R., Rosenthal, D. N., Dubin, A. M. 2012; 9 (12): 2010-2015

  Abstract

  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.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.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.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).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.

  View details for DOI 10.1016/j.hrthm.2012.08.031

  View details for Web of Science ID 000311791900029

  View details for PubMedID 23085485

• Are wide complex tachycardia algorithms applicable in children and patients with congenital heart disease? JOURNAL OF ELECTROCARDIOLOGY Ceresnak, S. R., Liberman, L., Avasarala, K., Tanel, R., Motonaga, K. S., Dubin, A. M. 2010; 43 (6): 694-700

  Abstract

  Several algorithms have been developed to help determine the etiology of wide complex tachycardias (WCTs) in adults. Sensitivity and specificity for differentiating supraventricular tachycardia (SVT) with aberration from ventricular tachycardia (VT) in adults have been demonstrated to be as high as 98% and 97%. These algorithms have not been tested in the pediatric population. We hypothesize that these algorithms have lower diagnostic accuracy in children and patients with congenital heart disease.A retrospective review of the pediatric electrophysiology database at Stanford from 2001 to 2008 was performed. All children with WCT, a 12-lead electrocardiogram (ECG) available for review, and an electrophysiology study confirming the etiology of the rhythm were included. Patients with a paced rhythm were excluded. The ECGs were analyzed by 2 electrophysiologists blinded to the diagnosis according to the algorithms described in Brugada et al,(2) and Vereckei et al.(5) Additional ECG findings were recorded by each electrophysiologist.A total of 65 WCT ECGs in 58 patients were identified. Supraventricular tachycardia was noted in 62% (40/65) and VT in 38% (25/65) of the ECGs. The mean age was 13.5 years (SD ± 5.1), the mean weight was 51.8 kg (SD ± 22.4), and 48% (31/65) were male. The mean tachycardia cycle length was 340 milliseconds (SD ± 95). Congenital heart disease (CHD) was present in 37% (24/65) of patients (7 tetralogy of Fallot, 6 Ebstein's, 4 double-outlet right ventricle, 3 complex CHD, 2 d-transposition of great arteries, 1 status-post orthotopic heart transplantation, 1 ventricular septal defect). The Brugada algorithm correctly predicted the diagnosis 69% (45/65) of the time, the Vereckei algorithm correctly predicted the diagnosis 66% (43/65) of the time, and the blinded reviewer correctly predicted the diagnosis 78% (51/65) of the time. There was no difference in the efficacy of the algorithms in patients with CHD vs those with structurally normal hearts. The findings of left superior axis deviation (P < .01) and a notch in the QRS downstroke of V(1) or V(2) (P < .01) were more common in VT than SVT, whereas a positive QRS deflection in V(1) (P = .03) was more commonly present in SVT than VT.The Brugada and Vereckei algorithms have lower diagnostic accuracy in the pediatric population and in patients with congenital heart disease than in the adult population. Left superior axis deviation and a notch in the QRS downstroke were more commonly associated with VT, whereas a positive QRS deflection in V(1) was more commonly associated with SVT in this population.

  View details for DOI 10.1016/j.jelectrocard.2010.02.008

  View details for Web of Science ID 000284514700039

  View details for PubMedID 20382398