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Loss of Ventricular Pre-excitation During Non-invasive Testing Does Not Exclude High-Risk Accessory Pathways: A Multicenter Study of WPW in Children

Escudero, Carolina A; Ceresnak, Scott R; Collins, Kathryn K; Pass, Robert H; Aziz, Peter F; Blaufox, Andrew D; Ortega, Michel Cabrera; Cannon, Bryan C; Cohen, Mitchell; Dechert, Brynn E; Dubin, Anne M; Motonaga, Kara S; Epstein, Michael R; Erickson, Christopher C; Fishberger, Steven B; Gates, Gregory J; Capone, Christine A; Nappo, Lynn; Kertesz, Naomi J; Kim, Jeffrey J; Valdes, Santiago O; Kubuš, Peter; Law, Ian H; Maldonado, Jennifer; Moore, Jeremy P; Perry, James C; Sanatani, Shubhayan; Seslar, Stephen P; Shetty, Ira; Zimmerman, Frank J; Skinner, Jonathan R; Marcondes, Luciana; Stephenson, Elizabeth A; Asakai, Hiroko; Tanel, Ronn E; Uzun, Orhan; Etheridge, Susan P; Janson, Christopher
BACKGROUND: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. OBJECTIVE: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. METHODS: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. RESULTS: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). CONCLUSION/CONCLUSIONS: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.
PMID: 32497761
ISSN: 1556-3871
CID: 4469352

A multicenter review of ablation in the aortic cusps in young people

Nguyen, Minh B; Ceresnak, Scott R; Janson, Christopher M; Fishberger, Steven B; Love, Barry A; Blaufox, Andrew D; Motonaga, Kara S; Dubin, Anne M; Nappo, Lynn; Pass, Robert H
BACKGROUND: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. METHODS: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. RESULTS:[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. CONCLUSION/CONCLUSIONS: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.
PMID: 28568013
ISSN: 1540-8159
CID: 3076402

Defibrillation Testing Strategies of Pediatric and Adult Congenital Electrophysiologists during ICD Implantation

Baysa, Sherrie Joy A; Olen, Melissa; Kanter, Ronald J; Fishberger, Steven B
BACKGROUND:Recently, there has been an increasing number of internal medicine-trained electrophysiologists who choose not to test for acute defibrillation efficacy during implantable cardioverter defibrillator (ICD) implantation. It is not known whether this same trend is seen in pediatric electrophysiologists, who care primarily for patients with congenital heart disease or primary electrical problems. METHODS:Through a 14-question survey created on Survey Monkey, we asked the members of the Pediatric and Adult Congenital Electrophysiology Society (PACES) for their approach to ICD implantation. In particular, respondents were asked for their individual practice preferences on testing for acute defibrillation efficacy, including methods used for testing. RESULTS:There were 108 survey respondents. Thirty-nine percent test acute defibrillation efficacy at all implants, while 46% test in most patients, barring any clinical concerns. Another 14% routinely test during initial ICD system implants but not during generator changes with existing leads. Less than 1% of respondents do not routinely test acute defibrillation efficacy. CONCLUSIONS:The practice preferences of pediatric electrophysiologists in evaluating for acute defibrillation efficacy during ICD implants are in contrast to the recent trend in their internal medicine-trained counterparts. More studies are needed to determine if practice changes should be considered in the pediatric and adult congenital patient population.
PMID: 27226114
ISSN: 1540-8159
CID: 3104062

Wolff-Parkinson-White Syndrome: A Stepwise Deterioration to Sudden Death

Olen, Melissa M; Baysa, Sherrie J; Rossi, Anthony; Kanter, Ronald J; Fishberger, Steven B
PMID: 26719390
ISSN: 1524-4539
CID: 2042402

Applying Cardiac Resynchronization Criteria to Pediatric Patients: Fitting a Square Peg into a Round Hole?

Fishberger, Steven B; Kanter, Ronald J
In 1839, Jan Evengelista Purkinje discovered a grayish material lining the ovine endocardium which we now call the purkinje network (1); in 1893, Wilhelm His discovered the penetrating A-V bundle that bears his name (2); and in 1906, Sunao Tarawa traced the entire specialized A-V conduction system (3). Ever since these seminal observations, investigators and clinicians have marched in lockstep in their appreciation for - what was once called - the 'myogenic theory' of the heartbeat. The fact that altered conduction could have adverse hemodynamic consequences was first suspected by Wiggers in 1925: '...where artificial contractions were induced showed without fail that this early phase of slower contraction is prolonged and that the tension develops more gradually...Expressed in another way, the pressure developed...is far less in the artificially than in the naturally elicited beats.'(4) Fast-forward 60 years, when investigators provided irrefutable evidence that pacing the right ventricle (and by inference, delaying activation of the left) resulted in load-independent reduction in all indices of left ventricular contraction and relaxation (5,6). Even more to the point, among patients having dilated cardiomyopathy and left bundle branch block (LBBB), Grines showed that LBBB-induced shortening of left ventricular diastolic filling time causes regional reduction in systolic septal motion, with resultant global reduction in left ventricular function (7)
PMID: 26011239
ISSN: 1540-8167
CID: 1603542

Success Rates in Pediatric WPW Ablation are Improved with Three-Dimensional Mapping Systems Compared with Fluoroscopy Alone: A Multicenter Study

Ceresnak, Scott R; Dubin, Anne M; Kim, Jeffrey J; Valdes, Santiago O; Fishberger, Steven B; Shetty, Ira; Zimmerman, Frank; Tanel, Ronn E; Epstein, Michael R; Motonaga, Kara S; Capone, Christine A; Nappo, Lynn; Gates, Gregory J; Pass, Robert H
INTRODUCTION: 3D mapping (3D) systems are frequently used for ablation of SVT. Prior studies have demonstrated radiation dosage reduction with 3D, but there are no data on whether 3D improves the efficacy of ablation of WPW. We sought to determine if 3D improves the success rate for ablation of WPW in children. METHODS: Multicenter retrospective study including patients
PMID: 25600208
ISSN: 1045-3873
CID: 1440002

Creation of Partial Fascicular Block: An Approach to Ablation of Idiopathic Left Ventricular Tachycardia in the Pediatric Population

Fishberger, Steven B; Olen, Melissa M; Rollinson, Nancy L; Rossi, Anthony F
BACKGROUND: Catheter ablation of idiopathic left ventricular tachycardia in the pediatric population remains challenging. A recent multicenter study reported limited success with 14% not undergoing ablation due to inability to induce ventricular tachycardia (VT) or blood pressure instability during tachycardia. Creating complete or partial fascicular block with radiofrequency catheter ablation is a technique that may eliminate VT. This approach is performed during sinus rhythm, enabling atrioventricular conduction monitoring and maintaining stable hemodynamics. Importantly, induction of VT is not necessary for mapping or assessing efficacy of the procedure. METHODS: A retrospective review of pediatric patients (3-17 years) with recurrent, documented idiopathic left ventricular tachycardia by electrocardiogram who received catheter ablation by creating fascicular block as a therapeutic endpoint was performed. All had ablation at the site of an identified Purkinje potential. RESULTS: There were six patients with idiopathic left ventricular tachycardia, five originating from the posterior fascicle and one from the anterior fascicle. VT was not induced or spontaneous in four patients using programmed stimulation and isoproterenol infusion. All patients had a QRS axis shift following ablation, though none met criteria for fascicular block. At follow up (7-49 months, mean 27 months), all patients had persistence of this shift. There were no recurrences of VT and none of the patients were taking antiarrhythmic medication. CONCLUSION: The technique of creating partial fascicular block appears to be a safe and effective approach to ablation of idiopathic left ventricular tachycardia in children.
PMID: 25469902
ISSN: 0147-8389
CID: 1371072

Lone atrial fibrillation in the young - perhaps not so "lone"?

Ceresnak, Scott R; Liberman, Leonardo; Silver, Eric S; Fishberger, Steven B; Gates, Gregory J; Nappo, Lynn; Mahgerefteh, Joseph; Pass, Robert H
OBJECTIVE: To determine if pediatric patients with a history of lone atrial fibrillation (AF) have other forms of supraventricular tachycardia (SVT) that may potentially trigger AF. STUDY DESIGN: A multicenter review of patients with lone AF who underwent electrophysiology (EP) study from 2006-2011 was performed. Inclusion criteria: age
PMID: 23092527
ISSN: 0022-3476
CID: 897282

Congenital long-QT syndrome in Addison's disease: a novel association [Case Report]

Lang, Sean M; Rollinson, Nancy L; Fishberger, Steven B
This report describes a teenager found to have both Addision's disease and long-QT syndrome type 1. This association is unique, but congenital long-QT channelopathies have been associated with other endocrinopathies. It remains to be seen whether genetic investigation should be performed for all patients with long-QTc's and endocrinopathies.
PMID: 22311567
ISSN: 0172-0643
CID: 897272

Percutaneous right ventricular support during catheter ablation of intra-atrial reentrant tachycardia in an adult with a mustard baffle--a novel use of the Impella device [Case Report]

Fishberger, Steven B; Asnes, Jeremy D; Rollinson, Nancy L; Cleman, Michael W
Late sequelea following a Mustard operation for transposition of the great arteries (TGA) include atrial arrhythmias and dysfunction of the systemic right ventricle. Catheter mapping and ablation of atrial tachycardia in the setting of significant right ventricular dysfunction may result in hemodynamic compromise. We report the novel use of the Impella percutaneous microaxial flow pump to support cardiac output in an adult patient with a Mustard operation for TGA who experienced a cardiac arrest during a prior ablation attempt. The Impella device was placed via a retrograde approach across the aortic valve into the right ventricle providing hemodynamic stability for successful mapping and ablation of intra-atrial reentrant tachycardia.
PMID: 20386974
ISSN: 1383-875x
CID: 897252