Susan Payne Etheridge

All Publications

• The expanding genetic, mechanistic, and phenotype scope of CACNA1C-mediated disease. Heart rhythm Landstrom, A. P., Etheridge, S. P. 2025; 22 (6): 1626-1627

  View details for DOI 10.1016/j.hrthm.2025.03.1991

  View details for PubMedID 40449979

• Children are Less Likely Than Adults to Develop Complete Heart Block Following TAVR. Pediatric cardiology Newlon, C. A., Niu, M. C., Binka, E., Boucek, D. M., Ou, Z., Etheridge, S. P., Pilcher, T. A., Martin, M. H., Gray, R. G., Asaki, S. Y. 2025

  Abstract

  TAVR is an alternative to surgical aortic valve [AoV] replacement. In adults, rates of atrioventricular block [AVB] requiring permanent pacemaker [PPM] placement in the modern era are 4-24%. Post-TAVR conduction abnormality incidence and risk factors are unknown in children and young adults. Describe post-TAVR conduction abnormalities in children and young adults. Retrospective single-center review of patients undergoing TAVR (9/2014 to 6/2021). Patients with pre-existing complete AVB or PPM were excluded (N=1). The relationship between described adult risk factors for AVB and primary outcome of new conduction abnormality was assessed. Of 28 cases (Ages 3.5-22 y), 50% were male and 43% had isolated AoV disease, and the remainder with multilevel obstructive left-sided heart disease (29%) or complex congenital heart disease (29%). Baseline conduction abnormality was present in 57% (16/28), with right bundle branch block the most prevalent (9, 56%). Post-TAVR, acute- and late-onset conduction abnormalities occurred in 9 and 2 patients, respectively, and resolved in 8/11 patients during follow-up. One patient required PPM for complete heart block. There was no association between new conduction abnormality and previously reported adult risk factors-baseline RBBB, membranous septum length, valve implantation depth, or degree of valve oversizing. There was no relationship between outcome and baseline conduction abnormality nor history of multiple AoV interventions. In our pediatric series, AVB requiring PPM was rare following TAVR, with an incidence 3.6%-lower than average rates reported in adult literature. We identified no association of conduction abnormality with described adult risk factors.

  View details for DOI 10.1007/s00246-025-03889-3

  View details for PubMedID 40360941

• Immune and nonimmune congenital heart block: A tale of two very different entities. Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology Etheridge, S., Strasburger, J. 2025

  View details for DOI 10.1093/europace/euaf041

  View details for PubMedID 40036946

• Children's Heartbeat: A Web-Based Program to Spread Knowledge on Arrhythmias in Children. Pediatric cardiology Gakenheimer-Smith, L., Etheridge, S. P., Batra, A. S., Sanatani, S., Holiman, H., Nahey, T., Balaji, S. 2024

  Abstract

  Expertise in pediatric arrhythmia management is lacking in most low- and middle- income countries (LMIC). Strategies to disseminate education in pediatric electrophysiology are essential to meet this need. Children's Heartbeat was created to meet the growing demand for pediatric electrophysiologic consultation in LMIC. Children's Heartbeat is a virtual video-conferencing program that uses the Extension for Community Healthcare Outcomes model to disseminate knowledge about pediatric arrhythmia management from pediatric electrophysiologists to clinicians in medically under-resourced regions worldwide. Monthly virtual case-based sessions are held with pediatric electrophysiologists and clinicians in medically under-resourced settings to discuss pediatric electrophysiology management. Since its inception, Children's Heartbeat viewership has grown exponentially to include 181 total registrants, 64 average monthly participants, and an additional 121 average viewers of recorded sessions. Attendees have expressed increased confidence in pediatric arrhythmia management. Children's Heartbeat has successfully provided pediatric electrophysiology consultation to medically under-resourced regions globally and have increased clinicians' confidence in caring for children with arrhythmias. Future directions include spreading the program to general pediatric and pediatric cardiology trainees and practicing pediatricians in rural parts of high-income countries and evaluating the direct impact of Children's Heartbeat on the management of arrhythmias in pediatric patients in LMIC.

  View details for DOI 10.1007/s00246-024-03635-1

  View details for PubMedID 39369164

• Implantable cardioverter defibrillators in pediatric patients: Yet another example of healthcare divergence? Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology DeWitt, E., Janousek, J., Etheridge, S. 2024

  View details for DOI 10.1093/europace/euae230

  View details for PubMedID 39228363

• Vigorous Exercise in Patients With Congenital Long-QT Syndrome: Results of the Prospective, Observational, Multinational LIVE-LQTS Study. Circulation Lampert, R., Day, S., Ainsworth, B., Burg, M., Marino, B. S., Salberg, L., Tome Esteban, M. T., Abrams, D. J., Aziz, P. F., Barth, C., Behr, E. R., Bell, C., Berul, C. I., Bos, J. M., Bradley, D., Cannom, D. S., Cannon, B. C., Concannon, M. A., Cerrone, M., Czosek, R. J., Dubin, A. M., Dziura, J., Erickson, C. C., Estes, N. A., Etheridge, S. P., Goldenberg, I., Gray, B., Haglund-Turnquist, C., Harmon, K., James, C. A., Johnsrude, C., Kannankeril, P., Lara, A., Law, I. H., Li, F., Link, M. S., Molossi, S. M., Olshansky, B., Noseworthy, P. A., Saarel, E. V., Sanatani, S., Shah, M., Simone, L., Skinner, J., Tomaselli, G. F., Ware, J. S., Webster, G., Zareba, W., Zipes, D. P., Ackerman, M. J. 2024

  Abstract

  Whether vigorous exercise increases risk of ventricular arrhythmias for individuals diagnosed and treated for congenital long-QT syndrome (LQTS) remains unknown.The National Institutes of Health-funded LIVE-LQTS study (Lifestyle and Exercise in Genetic Cardiovascular Conditions) prospectively enrolled individuals 8 to 60 years of age with phenotypic or genotypic LQTS from 37 sites in 5 countries from May 2015 to February 2019. Participants (or parents) answered physical activity and clinical events surveys every 6 months for 3 years with follow-up completed in February 2022. Vigorous exercise was defined as ≥6 metabolic equivalents for >60 hours per year. A blinded Clinical Events Committee adjudicated the composite end point of sudden death, sudden cardiac arrest, ventricular arrhythmia treated by an implantable cardioverter defibrillator, and likely arrhythmic syncope. A National Death Index search ascertained vital status for those with incomplete follow-up. A noninferiority hypothesis (boundary of 1.5) between vigorous exercisers and others was tested with multivariable Cox regression analysis.Among the 1413 participants (13% <18 years of age, 35% 18-25 years of age, 67% female, 25% with implantable cardioverter defibrillators, 90% genotype positive, and 49% with LQT1), 91% were treated with beta-blockers, left cardiac sympathetic denervation, or implantable cardioverter defibrillator; 52% participated in vigorous exercise (55% competitively). Thirty-seven individuals experienced the composite end point (including one sudden cardiac arrest and one sudden death in the nonvigorous group, one sudden cardiac arrest in the vigorous group) with overall event rates at 3 years of 2.6% in the vigorous and 2.7% in the nonvigorous exercise groups. The unadjusted hazard ratio for experience of events for the vigorous group compared with the nonvigorous group was 0.97 (90% CI, 0.57-1.67), with an adjusted hazard ratio of 1.17 (90% CI, 0.67-2.04). The upper 95% one-sided confidence level extended beyond the 1.5 boundary. Neither vigorous or nonvigorous exercise was found to be superior in any group or subgroup.Among individuals diagnosed with phenotypic or genotypic LQTS who were risk assessed and treated in experienced centers, LQTS-associated cardiac event rates were low and similar between those exercising vigorously and those not exercising vigorously. Consistent with the low event rate, CIs are wide, and noninferiority was not demonstrated. These data further inform shared decision-making discussions between patient and physician about exercise and competitive sports participation.URL: https://www.clinicaltrials.gov; Unique identifier: NCT02549664.

  View details for DOI 10.1161/CIRCULATIONAHA.123.067590

  View details for PubMedID 39051104

• Vigorous Exercise in Individuals With Long QT Syndrome (LQTS): Primary Results of the Prospective, Multinational Lifestyle and Exercise in LQTS (LIVE-LQTS) Study Lampert, R., Day, S., Ainsworth, B., Burg, M., Marino, B. S., Salberg, L., Tome, M., Abrams, D. J., Aziz, P. F., Barth, C., Bell, C., Berul, C. I., Bos, J. M., Bradley, D., Cannom, D. S., Cannon, B. C., Concannon, M. A., Cerrone, M., Czosek, R. J., Dubin, A. M., Dziura, J. D., Erickson, C. C., Estes, N. A., Etheridge, S. P., Gray, B., Haglund-Turnquist, C., James, C., Johnsrude, C., Kannankeril, P., Lara, A., Law, I. H., Li, F., Link, M. S., Molossi, S. M., Olshansky, B., Noseworthy, P. A., Saarel, E. V., Sanatani, S., Shah, M., Simone, L., Skinner, J., Tomaselli, G. F., Ware, J. S., Webster, G. G., Zipes, D. P., Ackerman, M. J. LIPPINCOTT WILLIAMS & WILKINS. 2023: E307

  View details for Web of Science ID 001297469300051

• 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

• Vigorous Exercise in Patients With Hypertrophic Cardiomyopathy. JAMA cardiology Lampert, R., Ackerman, M. J., Marino, B. S., Burg, M., Ainsworth, B., Salberg, L., Tome Esteban, M. T., Ho, C. Y., Abraham, R., Balaji, S., Barth, C., Berul, C. I., Bos, M., Cannom, D., Choudhury, L., Concannon, M., Cooper, R., Czosek, R. J., Dubin, A. M., Dziura, J., Eidem, B., Emery, M. S., Estes, N. A., Etheridge, S. P., Geske, J. B., Gray, B., Hall, K., Harmon, K. G., James, C. A., Lal, A. K., Law, I. H., Li, F., Link, M. S., McKenna, W. J., Molossi, S., Olshansky, B., Ommen, S. R., Saarel, E. V., Saberi, S., Simone, L., Tomaselli, G., Ware, J. S., Zipes, D. P., Day, S. M., LIVE Consortium, Abrahms, D., Ashley, E., Aziz, P., Batra, A., Cerrone, M., Colan, S., Erickson, C., Ferhaan, A., Gollob, M. J., Johnsrude, C., Kannankeril, P., Kanter, R., Li, W., Masri, A., Murphy, A., Nandi, D., Perez, M., Perry, J., Popjes, E., Rao, R., Rosenthal, D., Sanatani, S., Semsarian, C., Shah, M., Skinner, J., Tardif, J., Towbin, J., Turer, A., Webster, G., Wever-Pinzon, O., Wong, T. 2023

  Abstract

  Importance: Whether vigorous intensity exercise is associated with an increase in risk of ventricular arrhythmias in individuals with hypertrophic cardiomyopathy (HCM) is unknown.Objective: To determine whether engagement in vigorous exercise is associated with increased risk for ventricular arrhythmias and/or mortality in individuals with HCM. The a priori hypothesis was that participants engaging in vigorous activity were not more likely to have an arrhythmic event or die than those who reported nonvigorous activity.Design, Setting, and Participants: This was an investigator-initiated, prospective cohort study. Participants were enrolled from May 18, 2015, to April 25, 2019, with completion in February 28, 2022. Participants were categorized according to self-reported levels of physical activity: sedentary, moderate, or vigorous-intensity exercise. This was a multicenter, observational registry with recruitment at 42 high-volume HCM centers in the US and internationally; patients could also self-enroll through the central site. Individuals aged 8 to 60 years diagnosed with HCM or genotype positive without left ventricular hypertrophy (phenotype negative) without conditions precluding exercise were enrolled.Exposures: Amount and intensity of physical activity.Main Outcomes and Measures: The primary prespecified composite end point included death, resuscitated sudden cardiac arrest, arrhythmic syncope, and appropriate shock from an implantable cardioverter defibrillator. All outcome events were adjudicated by an events committee blinded to the patient's exercise category.Results: Among the 1660 total participants (mean [SD] age, 39 [15] years; 996 male [60%]), 252 (15%) were classified as sedentary, and 709 (43%) participated in moderate exercise. Among the 699 individuals (42%) who participated in vigorous-intensity exercise, 259 (37%) participated competitively. A total of 77 individuals (4.6%) reached the composite end point. These individuals included 44 (4.6%) of those classified as nonvigorous and 33 (4.7%) of those classified as vigorous, with corresponding rates of 15.3 and 15.9 per 1000 person-years, respectively. In multivariate Cox regression analysis of the primary composite end point, individuals engaging in vigorous exercise did not experience a higher rate of events compared with the nonvigorous group with an adjusted hazard ratio of 1.01. The upper 95% 1-sided confidence level was 1.48, which was below the prespecified boundary of 1.5 for noninferiority.Conclusions and Relevance: Results of this cohort study suggest that among individuals with HCM or those who are genotype positive/phenotype negative and are treated in experienced centers, those exercising vigorously did not experience a higher rate of death or life-threatening arrhythmias than those exercising moderately or those who were sedentary. These data may inform discussion between the patient and their expert clinician around exercise participation.

  View details for DOI 10.1001/jamacardio.2023.1042

  View details for PubMedID 37195701

• 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

• Virtual Transformation and the Use of Social Media: Cardiac Electrophysiology Education in the Post-COVID-19 Era CURRENT TREATMENT OPTIONS IN CARDIOVASCULAR MEDICINE Han, J. K., Baykaner, T., DeSimone, C., Etheridge, S. P., Futyma, P., Saha, S. A., Stiles, M. K., Gopinathannair, R., Kabra, R., Merchant, F. M. 2021; 23 (11): 70

  Abstract

  The COVID-19 pandemic has significantly impacted the delivery of education for all specialties, including cardiac electrophysiology. This review will provide an overview of the COVID-19 spurred digital transformation of electrophysiology education for practicing clinicians and trainees in electrophysiology and cover the use of social media in these educational efforts.Major international, national, and local meetings and electrophysiology fellowship-specific educational sessions have transitioned rapidly to virtual and distanced learning, enhanced by social media. This has allowed for participation in educational activities by electrophysiologists on a wider, more global scale. Social media has also allowed rapid dissemination of new advances, techniques, and research findings in real time and to a global audience, but caution must be exercised as pitfalls also exist.The digital and social media transformation of cardiac electrophysiology education has arrived and revolutionized the way education is delivered and consumed. Continued hybrid in-person and virtual modalities will provide electrophysiologists the flexibility to choose the best option to suit their individual needs and preferences for continuing education.

  View details for DOI 10.1007/s11936-021-00948-9

  View details for Web of Science ID 000708449000001

  View details for PubMedID 34690486

  View details for PubMedCentralID PMC8523345

• 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

• Management of Congenital Long-QT Syndrome: Commentary From the Experts. Circulation. Arrhythmia and electrophysiology Kaufman, E. S., Eckhardt, L. L., Ackerman, M. J., Aziz, P. F., Behr, E. R., Cerrone, M., Chung, M. K., Cutler, M. J., Etheridge, S. P., Krahn, A. D., Lubitz, S. A., Perez, M. V., Priori, S. G., Roberts, J. D., Roden, D. M., Schulze-Bahr, E., Schwartz, P. J., Shimizu, W., Shoemaker, M. B., Sy, R. W., Towbin, J. A., Viskin, S., A M Wilde, A., Zareba, W. 2021: CIRCEP120009726

  Abstract

  While published guidelines are useful in the care of patients with long-QT syndrome, it can be difficult to decide how to apply the guidelines to individual patients, particularly those with intermediate risk. We explored the diversity of opinion among 24 clinicians with expertise in long-QT syndrome. Experts from various regions and institutions were presented with 4 challenging clinical scenarios and asked to provide commentary emphasizing why they would make their treatment recommendations. All 24 authors were asked to vote on case-specific questions so as to demonstrate the degree of consensus or divergence of opinion. Of 24 authors, 23 voted and 1 abstained. Details of voting results with commentary are presented. There was consensus on several key points, particularly on the importance of the diagnostic evaluation and of beta-blocker use. There was diversity of opinion about the appropriate use of other therapeutic measures in intermediate-risk individuals. Significant gaps in knowledge were identified.

  View details for DOI 10.1161/CIRCEP.120.009726

  View details for PubMedID 34238011

• 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

• An International Multi-Center Evaluation of Type 5 Long QT Syndrome: A Low Penetrant Primary Arrhythmic Condition. Circulation Roberts, J. D., Asaki, S. Y., Mazzanti, A. n., Bos, J. M., Tuleta, I. n., Muir, A. R., Crotti, L. n., Krahn, A. D., Kutyifa, V. n., Shoemaker, M. B., Johnsrude, C. L., Aiba, T. n., Marcondes, L. n., Baban, A. n., Udupa, S. n., Dechert, B. n., Fischbach, P. n., Knight, L. M., Vittinghoff, E. n., Kukavica, D. n., Stallmeyer, B. n., Giudicessi, J. R., Spazzolini, C. n., Shimamoto, K. n., Tadros, R. n., Cadrin-Tourigny, J. n., Duff, H. J., Simpson, C. S., Roston, T. M., Wijeyeratne, Y. D., El Hajjaji, I. n., Yousif, M. D., Gula, L. J., Leong-Sit, P. n., Chavali, N. n., Landstrom, A. P., Marcus, G. M., Dittmann, S. n., Wilde, A. A., Behr, E. R., Tfelt-Hansen, J. n., Scheinman, M. M., Perez, M. V., Kaski, J. P., Gow, R. M., Drago, F. n., Aziz, P. F., Abrams, D. J., Gollob, M. H., Skinner, J. R., Shimizu, W. n., Kaufman, E. S., Roden, D. M., Zareba, W. n., Schwartz, P. J., Schulze-Bahr, E. n., Etheridge, S. P., Priori, S. G., Ackerman, M. J. 2020

  Abstract

  Background: Insight into type 5 long QT syndrome (LQT5) has been limited to case reports and small family series. Improved understanding of the clinical phenotype and genetic features associated with rare KCNE1 variants implicated in LQT5 was sought through an international multi-center collaboration. Methods: Patients with either presumed autosomal dominant LQT5 (N = 229) or the recessive Type 2 Jervell and Lange-Nielsen syndrome (JLNS2, N = 19) were enrolled from 22 genetic arrhythmia clinics and 4 registries from 9 countries. KCNE1 variants were evaluated for ECG penetrance (defined as QTc > 460ms on presenting ECG) and genotype-phenotype segregation. Multivariable Cox regression was used to compare the associations between clinical and genetic variables with a composite primary outcome of definite arrhythmic events, including appropriate implantable cardioverter-defibrillator shocks, aborted cardiac arrest, and sudden cardiac death. Results: A total of 32 distinct KCNE1 rare variants were identified in 89 probands and 140 genotype positive family members with presumed LQT5 and an additional 19 JLNS2 patients. Among presumed LQT5 patients, the mean QTc on presenting ECG was significantly longer in probands (476.9 ± 38.6ms) compared to genotype positive family members (441.8 ± 30.9ms, p<0.001). ECG penetrance for heterozygous genotype positive family members was 20.7% (29/140). A definite arrhythmic event was experienced in 16.9% (15/89) of heterozygous probands in comparison with 1.4% (2/140) of family members (adjusted hazard ratio [HR]: 11.6, 95% confidence interval [CI]: 2.6-52.2; p=0.001). Event incidence did not differ significantly for JLNS2 patients relative to the overall heterozygous cohort (10.5% [2/19]; HR: 1.7, 95% CI: 0.3-10.8, p=0.590). The cumulative prevalence of the 32 KCNE1 variants in the Genome Aggregation Database (gnomAD), which is a human database of exome and genome sequencing data from now over 140,000 individuals, was 238-fold greater than the anticipated prevalence of all LQT5 combined (0.238% vs. 0.001%). Conclusions: The present study suggests that putative/confirmed loss-of-function KCNE1 variants predispose to QT-prolongation, however the low ECG penetrance observed suggests they do not manifest clinically in the majority of individuals, aligning with the mild phenotype observed for JLNS2 patients.

  View details for DOI 10.1161/CIRCULATIONAHA.119.043114

  View details for PubMedID 31941373

• 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

• 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

• Atrioventricular block after congenital heart surgery: Analysis from the Pediatric Cardiac Critical Care Consortium Romer, A. J., Tabbutt, S., Etheridge, S. P., Fischbach, P., Ghanayem, N. S., Reddy, V., Sahulee, R., Tanel, R. E., Tweddell, J. S., Gaies, M., Banerjee, M., Retzloff, L., Zhang, W., Patel, A. R. MOSBY-ELSEVIER. 2019: 1168-+

  View details for DOI 10.1016/j.jtcvs.2018.09.142

  View details for Web of Science ID 000458822000114

• Atrioventricular block after congenital heart surgery: Analysis from the Pediatric Cardiac Critical Care Consortium. The Journal of thoracic and cardiovascular surgery Romer, A. J., Tabbutt, S., Etheridge, S. P., Fischbach, P., Ghanayem, N. S., Reddy, V. M., Sahulee, R., Tanel, R. E., Tweddell, J. S., Gaies, M., Banerjee, M., Retzloff, L., Zhang, W., Patel, A. R. 2019; 157 (3): 1168-1177.e2

  Abstract

  Our primary aims were to describe the contemporary epidemiology of postoperative high-grade atrioventricular block (AVB), the timing of recovery and permanent pacemaker (PPM) placement, and to determine predictors for development of and recovery from AVB.Patients who underwent congenital heart surgery from August 2014 to June 2017 were analyzed for AVB using the Pediatric Cardiac Critical Care Consortium registry. Predictors of AVB with or without PPM were identified using multinomial logistic regression. We used these predictors to model the probability of PPM for the subgroup of patients with intraoperative complete AVB.We analyzed 15,901 surgical hospitalizations; 422 (2.7%) were complicated by AVB and 162 (1.0%) patients underwent PPM placement. In patients with transient AVB, 50% resolved by 2 days, and 94% resolved by 10 days. In patients who received a PPM, 50% were placed by 8 days and 62% were placed by 10 days. Independent risk factors associated with PPM compared with resolution of AVB were longer duration of cardiopulmonary bypass (relative risk ratio, 1.04; P = .023) and a high-risk operation (relative risk ratio, 2.59; P < .001). Among patients with complete AVB originating in the operating room, those with the highest predicted probability of PPM had a PPM placed only 77% of the time.In this cohort, postoperative AVB complicated almost 3% of congenital heart surgery cases and 1% of patients underwent PPM placement. Because almost all patients (94%) with transient AVB had resolution by 10 days, our results suggest there is limited benefit to delaying PPM placement beyond that time frame.

  View details for DOI 10.1016/j.jtcvs.2018.09.142

  View details for PubMedID 30917883

• 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

• PACES/HRS Expert Consensus Statement on the Evaluation and Management of Ventricular Arrhythmias in the Child With a Structurally Normal Heart. Heart rhythm Crosson, J. E., Callans, D. J., Bradley, D. J., Dubin, A., Epstein, M., Etheridge, S., Papez, A., Phillips, J. R., Rhodes, L. A., Saul, P., Stephenson, E., Stevenson, W., Zimmerman, F. 2014; 11 (9): e55-78

  View details for DOI 10.1016/j.hrthm.2014.05.010

  View details for PubMedID 24814375

• Pediatric Nonpost-Operative Junctional Ectopic Tachycardia Medical Management and Interventional Therapies JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Collins, K. K., Van Hare, G. F., Kertesz, N. J., Law, I. H., Bar-Cohen, Y., Dubin, A. M., Etheridge, S. P., Berul, C. I., Avari, J. N., Tuzcu, V., Sreeram, N., Schaffer, M. S., Fournier, A., Sanatani, S., Snyder, C. S., Smith, R. T., Arabia, L., Hamilton, R., Chun, T., Liberman, L., Kakavand, B., Paul, T., Tanel, R. E. 2009; 53 (8): 690-697

  Abstract

  To determine the outcomes of medical management, pacing, and catheter ablation for the treatment of nonpost-operative junctional ectopic tachycardia (JET) in a pediatric population.Nonpost-operative JET is a rare tachyarrhythmia that is associated with a high rate of morbidity and mortality. Most reports of clinical outcomes were published before the routine use of amiodarone or ablation therapies.This is an international, multicenter retrospective outcome study of pediatric patients treated for nonpost-operative JET.A total of 94 patients with JET and 5 patients with accelerated junctional rhythm (age 0.8 year, range fetus to 16 years) from 22 institutions were identified. JET patients presenting at age < or =6 months were more likely to have incessant JET and to have faster JET rates. Antiarrhythmic medications were utilized in a majority of JET patients (89%), and of those, amiodarone was the most commonly reported effective agent (60%). Radiofrequency ablation was conducted in 17 patients and cryoablation in 27, with comparable success rates (82% radiofrequency vs. 85% cryoablation, p = 1.0). Atrioventricular junction ablation was required in 3% and pacemaker implantation in 14%. There were 4 (4%) deaths, all in patients presenting at age < or =6 months.Patients with nonpost-operative JET have a wide range of clinical presentations, with younger patients demonstrating higher morbidity and mortality. With current medical, ablative, and device therapies, the majority of patients have a good clinical outcome.

  View details for DOI 10.1016/j.jacc.2008.11.019

  View details for Web of Science ID 000263666800008

  View details for PubMedID 19232902

• The use of Implantable cardioverter-defibrillators in pediatric patients awaiting heart transplantation JOURNAL OF CARDIAC FAILURE Dubin, A. M., Berul, C. I., Bevilacqua, L. M., Collins, K. K., Etheridge, S. P., Fenrich, A. L., Friedman, R. A., Hamilton, R. M., Schaffer, M. S., Shah, M., Silka, M. J., Van Hare, G. F., Kertesz, N. J. 2003; 9 (5): 375-379

  Abstract

  This multicenter study evaluated experience with implantable cardioverter defibrillators (ICD) as a bridge to orthotopic heart transplantation (OHT) in children.The application of ICD therapy continues to expand in pediatric populations, due in part to improved technology and new indications, including the prevention of sudden death while awaiting OHT.We performed a retrospective review of ICD databases at 9 pediatric transplant centers.Twenty-eight patients (16 males) underwent implantation or had a preexisting ICD while awaiting OHT between 1990 and 2002. The median age at implant was 14.3 years (11 months to 21 years) with a median weight of 49 kg (11.7-88 kg). Diagnoses included cardiomyopathy (n=22), and congenital heart disease (n=6). Indications for ICD implantation included ventricular tachycardia/fibrillation (n=23), syncope (n=5), aborted sudden death with no documentation of rhythm disturbance (n=5), ventricular ectopy (n=1), and poor function (n=5). Of the 28 ICDs, 23 were implanted by a transvenous approach and 5 by epicardial route. There were 55 defibrillator discharges in 17 patients, 47 (85%) of which (in 13 patients) were appropriate. The 8 inappropriate discharges (in 6 patients) were triggered by sinus tachycardia, inappropriate sensing, and atrial flutter. The mean time from implantation to first appropriate shock was 6.9 months (1 day to 2.6 years). Twenty-one patients underwent transplantation during the study period, whereas 2 died while awaiting a donor. Morbidity included a lead fracture, 3 episodes of electromechanical dissociation, and 1 episode of electrical storm.ICD implantation represents an effective bridge to transplantation in pediatric patients. The complication rate is low, with inappropriate device discharge due primarily to sinus tachycardia or atrial flutter. There is a high incidence of appropriate ICD therapy for malignant ventricular arrhythmias in this highly selected group of patients.

  View details for DOI 10.1054/s1071-9164(03)00128-3

  View details for Web of Science ID 000186326500006

  View details for PubMedID 14583898