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We present an unusual case of alternating left anterior and left posterior fascicular block. Given the known risk for progression to complete atrioventricular block with alternating right bundle and left bundle branch block, we performed an electrophysiological study. Findings were consistent with infra-Hisian disease, and the patient underwent pacemaker implantation.
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INTRODUCTION: Artificial intelligence (AI) ECG arrhythmia mapping provides arrhythmia source localization using 12-lead ECG data; whether this information impacts procedural efficiency is unknown. We performed a retrospective, case-control study to evaluate the hypothesis that AI ECG mapping may reduce time to ablation, procedural duration, and fluoroscopy. MATERIALS AND METHODS: Cases in which system output was used were retrospectively enrolled according to IRB-approved protocols at each site. Matched control cases were enrolled in reverse chronological order beginning on the last day for which the technology was unavailable. Controls were matched based upon physician, institution, arrhythmia, and a predetermined complexity rating. Procedural metrics, fluoroscopy data, and clinical outcomes were assessed from time-stamped medical records. RESULTS: The study group consisted of 28 patients (age 65 ± 11 years, 46% female, left atrial dimension 4.1 ± 0.9 cm, LVEF 50 ± 18%) and was similar to 28 controls. The most common arrhythmia types were atrial fibrillation (n = 10), premature ventricular complexes (n = 8), and ventricular tachycardia (n = 6). Use of the system was associated with a 19.0% reduction in time to ablation (133 ± 48 vs. 165 ± 49 min, p = 0.02), a 22.6% reduction in procedure duration (233 ± 51 vs. 301 ± 83 min, p < 0.001), and a 43.7% reduction in fluoroscopy (18.7 ± 13.3 vs. 33.2 ± 18.0 min, p < 0.001) versus controls. At 6 months follow-up, arrhythmia-free survival was 73.5% in the study group and 63.3% in the control group (p = 0.56). CONCLUSION: Use of forward-solution AI ECG mapping is associated with reductions in time to first ablation, procedure duration, and fluoroscopy without an adverse impact on procedure outcomes or complications.
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Potenciales de Acción , Arritmias Cardíacas , Inteligencia Artificial , Ablación por Catéter , Valor Predictivo de las Pruebas , Tiempo de Tratamiento , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Arritmias Cardíacas/fisiopatología , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/cirugía , Ablación por Catéter/efectos adversos , Electrocardiografía , Técnicas Electrofisiológicas Cardíacas , Fluoroscopía , Frecuencia Cardíaca , Tempo Operativo , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento , Estudios de Casos y ControlesRESUMEN
BACKGROUND: Pulsed field ablation (PFA) induces cell death through electroporation using ultrarapid electrical pulses. We sought to compare the procedural efficiency characteristics, safety, and efficacy of ablation of atrial fibrillation (AF) using PFA compared with thermal energy ablation. METHODS: We performed an extensive literature search and systematic review of studies that compared ablation of AF with PFA versus thermal energy sources. Risk ratio (RR) 95% confidence intervals (CI) were measured for dichotomous variables and mean difference (MD) 95% CI were measured for continuous variables, where RR < 1 and MD < 0 favor the PFA group. RESULTS: We included 6 comparative studies for a total of 1012 patients who underwent ablation of AF: 43.6% with PFA (n = 441) and 56.4% (n = 571) with thermal energy sources. There were significantly shorter procedures times with PFA despite a protocolized 20-min dwell time (MD - 21.95, 95% CI - 33.77, - 10.14, p = 0.0003), but with significantly longer fluroscopy time (MD 5.71, 95% CI 1.13, 10.30, p = 0.01). There were no statistically significant differences in periprocedural complications (RR 1.20, 95% CI 0.59-2.44) or recurrence of atrial tachyarrhythmias (RR 0.64, 95% CI 0.31, 1.34) between the PFA and thermal ablation cohorts. CONCLUSIONS: Based on the results of this meta-analysis, PFA was associated with shorter procedural times and longer fluoroscopy times, but no difference in periprocedural complications or rates of recurrent AF when compared to ablation with thermal energy sources. However, larger randomized control trials are needed.
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Fibrilación Atrial , Ablación por Catéter , Venas Pulmonares , Humanos , Fibrilación Atrial/cirugía , Ablación por Catéter/métodos , Atrios Cardíacos/cirugía , Resultado del Tratamiento , Venas Pulmonares/cirugíaRESUMEN
Background: Pulsed field ablation (PFA) has emerged as a novel energy source for the ablation of atrial fibrillation (AF) using ultrarapid electrical pulses to induce cell death via electroporation. Objective: The purpose of this study was to compare the safety and acute efficacy of ablation for AF with PFA vs thermal energy sources. Methods: We performed an extensive literature search and systematic review of studies that evaluated the safety and efficacy of ablation for AF with PFA and compared them to landmark clinical trials for ablation of AF with thermal energy sources. Freeman-Tukey double arcsine transformation was used to establish variance of raw proportions followed by the inverse with the random-effects model to combine the transformed proportions and generate the pooled prevalence and 95% confidence interval (CI). Results: We included 24 studies for a total of 5203 patients who underwent AF ablation. Among these patients, 54.6% (n = 2842) underwent PFA and 45.4% (n = 2361) underwent thermal ablation. There were significantly fewer periprocedural complications in the PFA group (2.05%; 95% CI 0.94-3.46) compared to the thermal ablation group (7.75%; 95% CI 5.40-10.47) (P = .001). When comparing AF recurrence up to 1 year, there was a statistically insignificant trend toward a lower prevalence of recurrence in the PFA group (14.24%; 95% CI 6.97-23.35) compared to the thermal ablation group (25.98%; 95% CI 15.75-37.68) (P = .132). Conclusion: Based on the results of this meta-analysis, PFA was associated with lower rates of periprocedural complications and similar rates of acute procedural success and recurrent AF with up to 1 year of follow-up compared to ablation with thermal energy sources.
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BACKGROUND: Catheter ablation improves outcomes in symptomatic atrial fibrillation (AF) patients. However, its safety and efficacy in the very elderly (≥80 years old) is not well described. HYPOTHESIS: Ablation of AF in the very elderly is safe and effective. METHODS: We performed a retrospective study of all patients who underwent catheter ablation enrolled in the University of California, San Diego AF Ablation Registry. The primary outcome was freedom from atrial arrhythmias on or off antiarrhythmic drugs (AADs). RESULTS: Of 847 patients, 42 (5.0%) were 80 years of age or greater with a median age of 81.5 (80-82.3) and 805 (95.0%) were less than 80 years of age with a median age of 64.4 (57.6-70.2). Among those who were ≥80 years old, 29 were undergoing de novo ablation (69.0%), whereas in the younger cohort, 518 (64.5%) were undergoing de novo ablation (p = .548). There were no statistically significant differences in fluoroscopy (p = .406) or total procedure times (p = .076), AAD use (p = .611), or procedural complications (p = .500) between groups. After multivariable adjustment, there were no statistically significant differences in recurrence of any atrial arrhythmias on or off AAD (adjusted hazard ratio [AHR]: 0.75; 95% confidence interval [CI]: 0.45-1.23; p = .252), all-cause hospitalizations (AHR: 0.86; 95% CI: 0.46-1.60; p = .626), or all-cause mortality (AHR: 4.48; 95% CI: 0.59-34.07; p = .147) between the very elderly and the younger cohort. CONCLUSION: In this registry analysis, catheter ablation of AF appears similarly effective and safe in patients 80 years or older when compared to a younger cohort.
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Fibrilación Atrial , Ablación por Catéter , Humanos , Anciano , Anciano de 80 o más Años , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/cirugía , Fibrilación Atrial/complicaciones , Estudios Retrospectivos , Resultado del Tratamiento , Antiarrítmicos/uso terapéutico , Sistema de Registros , Ablación por Catéter/efectos adversos , Ablación por Catéter/métodos , RecurrenciaRESUMEN
BACKGROUND: High-dose isoproterenol infusion is a useful provocative maneuver to elicit triggers of atrial fibrillation (AF) during ablation. We evaluated whether the use of isoproterenol infusion to elicit triggers of AF after ablation is associated with differential outcomes. METHODS: We performed a retrospective study of all patients who underwent de novo radiofrequency catheter ablation of AF enrolled in the University of California, San Diego AF Ablation Registry. The primary outcome was freedom from atrial arrhythmias on or off antiarrhythmic drugs (AAD). RESULTS: Of 314 patients undergoing AF ablation, 235 (74.8%) received isoproterenol while 79 (25.2%) did not. Among those who received isoproterenol, 11 (4.7%) had additional triggers identified. There were no statistically significant differences in procedure time (p = 0.432), antiarrhythmic drug use (p = 0.289), procedural complications (p = 0.279), recurrences of atrial arrhythmias on or off AAD [adjusted hazard ratio (AHR) 0.92 (95% CI 0.58-1.46); p = 0.714], all-cause hospitalizations [AHR 1.00 (95% CI 0.60-1.67); p = 0.986], or all-cause mortality [AHR 0.14 (95% CI 0.01-3.52); p = 0.229] between groups. CONCLUSIONS: In this registry analysis, use of isoproterenol is safe but was not associated with a reduction in recurrence of atrial arrhythmias.
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Fibrilación Atrial , Ablación por Catéter , Humanos , Isoproterenol , Resultado del Tratamiento , Estudios Retrospectivos , Antiarrítmicos/uso terapéutico , Sistema de Registros , Ablación por Catéter/métodos , RecurrenciaRESUMEN
BACKGROUND: The accuracy of noninvasive arrhythmia source localization using a forward-solution computational mapping system has not yet been evaluated in blinded, multicenter analysis. This study tested the hypothesis that a computational mapping system incorporating a comprehensive arrhythmia simulation library would provide accurate localization of the site-of-origin for atrial and ventricular arrhythmias and pacing using 12-lead ECG data when compared with the gold standard of invasive electrophysiology study and ablation. METHODS: The VMAP study (Vectorcardiographic Mapping of Arrhythmogenic Probability) was a blinded, multicenter evaluation with final data analysis performed by an independent core laboratory. Eligible episodes included atrial and ventricular: tachycardia, fibrillation, pacing, premature atrial and ventricular complexes, and orthodromic atrioventricular reentrant tachycardia. Mapping system results were compared with the gold standard site of successful ablation or pacing during electrophysiology study and ablation. Mapping time was assessed from time-stamped logs. Prespecified performance goals were used for statistical comparisons. RESULTS: A total of 255 episodes from 225 patients were enrolled from 4 centers. Regional accuracy for ventricular tachycardia and premature ventricular complexes in patients without significant structural heart disease (n=75, primary end point) was 98.7% (95% CI, 96.0%-100%; P<0.001 to reject predefined H0 <0.80). Regional accuracy for all episodes (secondary end point 1) was 96.9% (95% CI, 94.7%-99.0%; P<0.001 to reject predefined H0 <0.75). Accuracy for the exact or neighboring segment for all episodes (secondary end point 2) was 97.3% (95% CI, 95.2%-99.3%; P<0.001 to reject predefined H0 <0.70). Median spatial accuracy was 15 mm (n=255, interquartile range, 7-25 mm). The mapping process was completed in a median of 0.8 minutes (interquartile range, 0.4-1.4 minutes). CONCLUSIONS: Computational ECG mapping using a forward-solution approach exceeded prespecified accuracy goals for arrhythmia and pacing localization. Spatial accuracy analysis demonstrated clinically actionable results. This rapid, noninvasive mapping technology may facilitate catheter-based and noninvasive targeted arrhythmia therapies. REGISTRATION: URL: https://www. CLINICALTRIALS: gov; Unique identifier: NCT04559061.
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Ablación por Catéter , Taquicardia Supraventricular , Taquicardia Ventricular , Complejos Prematuros Ventriculares , Ablación por Catéter/métodos , Electrocardiografía/métodos , Humanos , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/cirugíaAsunto(s)
Enfermedad de la Arteria Coronaria , Intervención Coronaria Percutánea , Puente de Arteria Coronaria , Enfermedad de la Arteria Coronaria/cirugía , Humanos , Revascularización Miocárdica , Grupo de Atención al Paciente , Intervención Coronaria Percutánea/efectos adversos , Resultado del TratamientoRESUMEN
BACKGROUND: The mechanisms for atrial fibrillation (AF) recurrence after pulmonary vein isolation (PVI) catheter ablation are unclear. Non-PV organized atrial arrhythmias (PAC, AT, macro-reentrant AFL) are possible contributors; however the prevalence and effect of their ablation on recurrent AF are unknown. We hypothesize that the identification and ablation of non-PV organized atrial arrhythmias were associated with less AF recurrence. METHODS: Patients who underwent repeat ablation for recurrent AF after prior PVI were retrospectively enrolled. The prevalence and characteristics of PV reconnections and non-PV organized atrial arrhythmias were identified. The outcomes of time to clinical AF recurrence, heart failure (HF) hospitalization, and mortality were analyzed in patients using multivariable adjusted Cox regression. RESULTS: In 74 patients with recurrent AF (age 66 ± 9 years, left atrial volume index 38 ± 10 ml/m2, 59% persistent AF), PV reconnections were found in 46 patients (61%), macro-reentrant atrial flutter in 27 patients (36%), and focal tachycardia in 12 patients (16%). Mapping and ablation of non-PV organized atrial arrhythmias were associated with a reduced recurrence of late clinical AF (adjusted HR 0.26, CI 0.08-0.85, p = 0.03) and the composite outcome of recurrence of late AF, HF hospitalization, and mortality (adjusted HR 0.38, CI 0.17-0.85, p = 0.02), with median follow-up of 1.6 (IQR 0.7-6.3) years. The presence of PV reconnections or empiric linear ablation was not associated with reduction in clinical AF or composite endpoints. CONCLUSION: The ablation of non-PV organized atrial arrhythmias resulted in a reduction of late clinical AF recurrence and composite outcome. In this challenging population, alternate mechanisms beyond PV reconnections need to be considered. Prospective studies are needed.
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Fibrilación Atrial , Ablación por Catéter , Venas Pulmonares , Anciano , Fibrilación Atrial/epidemiología , Ablación por Catéter/métodos , Humanos , Persona de Mediana Edad , Venas Pulmonares/cirugía , Recurrencia , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
PURPOSE: Early recurrence of atrial tachyarrhythmia (ER) is predictive of late recurrence of atrial tachyarrhythmia (LR) after first-time atrial fibrillation (AF) ablation, but the association in patients undergoing repeat AF ablation is unknown. We aim to determine the incidence and prognostic significance of ER after repeat ablation. METHODS: A total of 259 consecutive patients (mean age 64 years, 75.3% male) undergoing repeat AF ablation with complete follow-up data were included at a single institution from 2010 to 2015. ER and LR were defined as atrial tachyarrhythmia (AF, atrial flutter or atrial tachycardia) > 30 s within the 3-month blanking period (BP) and after the 3-month BP, respectively. RESULTS: ER occurred in 79/259 (30.5%), and LR occurred in 138/259 (53%) at a median follow-up of 1221 (IQR: 523-1712) days. Four-year freedom from LR was 22% and 56% in patients with and without ER, respectively (p < 0.001). After multivariate adjustment, ER was strongly associated with LR, cardioversion post BP, and repeat ablation, but not associated with hospitalization. Compared to those with no ER, there was a higher risk of LR when ER occurred within the first month of the BP [month 1: hazard ratio (HR) 2.32, confidence interval (CI) 1.57-3.74, p < 0.001; month 2: HR 2.01, CI 1.13-3.83, p = 0.02; month 3: HR 1.46, CI 0.5-3.36, p = 0.37], however the prediction of LR based on timing within the BP was poor (area under curve 0.64). CONCLUSION: Following repeat AF ablation, ER is strongly associated with LR, cardioversion post BP, and repeat ablation.
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Fibrilación Atrial , Ablación por Catéter , Venas Pulmonares , Fibrilación Atrial/epidemiología , Ablación por Catéter/efectos adversos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Venas Pulmonares/cirugía , Recurrencia , Taquicardia/cirugía , Resultado del TratamientoRESUMEN
PURPOSE: Mitral annular flutter (MAF) is a common arrhythmia after atrial fibrillation ablation. We sought to compare the efficacy and safety of catheter ablation utilizing either a left atrial anterior wall (LAAW) line or a lateral mitral isthmus (LMI) line. METHODS: We performed a systematic review for all studies that compared LAAW versus LMI lines. Risk ratio (RR) and mean difference (MD) 95% confidence intervals were measured for dichotomous and continuous variables, respectively. RESULTS: Four studies with a total of 594 patients were included, one of which was a randomized control trial. In the LMI ablation group, 40% of patients required CS ablation. There were no significant differences in bidirectional block (RR 1.26; 95% CI, 0.94-1.69) or ablation time (MD -1.5; 95% CI, -6.11-3.11), but LAAW ablation was associated with longer ablation line length (MD 11.42; 95% CI, 10.69-12.14) and longer LAA activation delay (MD 67.68; 95% CI, 33.47-101.89.14) when compared to LMI. There was no significant difference in pericardial effusions (RR 0.36; 95% CI, 0.39-20.75) between groups and more patients were maintained sinus rhythm (RR 1.19; 95% CI, 1.03-1.37, p = 0.02) who underwent LAAW compared to LMI. CONCLUSION: Ablation of mitral annular flutter with a LAAW line compared to a LMI line showed no difference in rates of acute bidirectional block, ablation time, or pericardial effusion. However, LAAW ablation required a longer ablation line length, resulted in greater LAA activation delayed and was associated with more sinus rhythm maintenance, with the added advantage of avoiding ablation in the CS.
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Apéndice Atrial , Fibrilación Atrial , Aleteo Atrial , Ablación por Catéter , Fibrilación Atrial/diagnóstico por imagen , Fibrilación Atrial/cirugía , Aleteo Atrial/diagnóstico por imagen , Aleteo Atrial/cirugía , Atrios Cardíacos/cirugía , Humanos , Válvula Mitral/diagnóstico por imagen , Válvula Mitral/cirugía , Ensayos Clínicos Controlados Aleatorios como Asunto , Resultado del TratamientoRESUMEN
In this review, we emphasize the unique value of recording the activation sequence of the His bundle or right bundle branch (RB) for diagnoses of various supraventricular and fascicular tachycardias. A close analysis of the His to RB (H-RB) activation sequence can help differentiate various forms of supraventricular tachycardias, namely atrioventricular nodal reentry tachycardia from concealed nodofascicular tachycardia, a common clinical dilemma. Furthermore, bundle branch reentry tachycardia and fascicular tachycardias often are included in the differential diagnosis of supraventricular tachycardia with aberrancy, and the use of this technique can help the operator make the distinction between supraventricular tachycardias and these other forms of ventricular tachycardias using the His-Purkinje system. We show that this technique is enhanced by the use of multipolar catheters placed to span the proximal His to RB position to record the activation sequence between proximal His potential to the distal RB potential. This allows the operator to fully analyze the activation sequence in sinus rhythm as compared to that during tachycardia and may help target ablation of these arrhythmias. We argue that 3 patterns of H-RB activation are commonly identified-the anterograde H-RB pattern, the retrograde H-RB (right bundle to His bundle) pattern, and the chevron H-RB pattern (simultaneous proximal His and proximal RB activation)-and specific arrhythmias tend to be associated with specific H-RB activation sequences. We show that being able to record and categorize this H-RB relationship can be instrumental to the operator, along with standard pacing maneuvers, to make an arrhythmia diagnosis in complex tachycardia circuits. We highlight the importance of H-RB activation patterns in these complex tachycardias by means of case illustrations from our groups as well as from prior reports.
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Fascículo Atrioventricular/fisiopatología , Estimulación Cardíaca Artificial/métodos , Electrocardiografía/métodos , Taquicardia Paroxística/diagnóstico , Taquicardia Ventricular/diagnóstico , Humanos , Taquicardia Paroxística/fisiopatología , Taquicardia Paroxística/terapia , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/terapiaRESUMEN
BACKGROUND: Stereotactic ablative radiotherapy (SAbR) is an emerging therapy for refractory ventricular tachycardia (VT). However, the current workflow is complicated, and the precision and safety in patients with significant cardiorespiratory motion and VT targets near the stomach may be suboptimal. OBJECTIVE: We hypothesized that automated 12-lead electrocardiogram (ECG) mapping and respiratory-gated therapy may improve the ease and precision of SAbR planning and facilitate safe radiation delivery in patients with refractory VT. METHODS: Consecutive patients with refractory VT were studied at 2 hospitals. VT exit sites were localized using a 3-D computational ECG algorithm noninvasively and compared to available prior invasive mapping. Radiotherapy (25 Gy) was delivered at end-expiration when cardiac respiratory motion was ≥0.6 cm or targets were ≤2 cm from the stomach. RESULTS: In 6 patients (ejection fraction 29% ± 13%), 4.2 ± 2.3 VT morphologies per patient were mapped. Overall, 7 out of 7 computational ECG mappings (100%) colocalized to the identical cardiac segment when prior invasive electrophysiology study was available. Respiratory gating was associated with smaller planning target volumes compared to nongated volumes (71 ± 7 vs 153 ± 35 cc, P < .01). In 2 patients with inferior wall VT targets close to the stomach (6 mm proximity) or significant respiratory motion (22 mm excursion), no GI complications were observed at 9- and 12-month follow-up. Implantable cardioverter-defibrillator shocks decreased from 23 ± 12 shocks/patient to 0.67 ± 1.0 (P < .001) post-SAbR at 6.0 ± 4.9 months follow-up. CONCLUSIONS: A workflow including computational ECG mapping and protocol-guided respiratory gating is feasible, is safe, and may improve the ease of SAbR planning. Studies to validate this workflow in larger populations are required.
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PURPOSE: To evaluate if specific AADs prescribed in the blanking period (BP) after catheter ablation of atrial fibrillation (AF) may be associated with reduced risk of early recurrence (ER) and/or late recurrence (LR) of atrial arrhythmias. METHODS: A total of 478 patients undergoing first-time ablation at a single institution were included. Outcomes were: ER, LR, discontinuation of AAD less than 90 days post-ablation, and second ablation. ER was defined as AF, atrial flutter (AFL), or atrial tachycardia (AT) > 30 seconds within BP. LR was defined as AF/AFL/AT > 30 seconds after BP. RESULTS: Of 478 patients, 14.9% were prescribed no AAD, 26.4% propafenone/flecainide, 34.5% sotalol/dofetilide, 10.7% dronedarone, and 13.6% amiodarone. Patients prescribed amiodarone were more likely to have persistent AF, hypertension, diabetes, and other comorbidities. In unadjusted analyses, there were no differences between groups in relation to ER (log rank P = 0.171), discontinuation of AAD before ninety days post-ablation (log rank P = 0.235), or freedom from second ablation (log rank P = 0.147). After multivariable adjustment, patients prescribed amiodarone or dronedarone were more likely to experience LR than those prescribed no AAD [Adjusted Hazard Ratio (AHR) 1.83, 95% CI 1.10-3.04, p = 0.02; AHR 1.79, 95% CI 1.05-3.05, p = 0.03, respectively]. CONCLUSION: Following first-time catheter ablation, there were no differences between specific AAD prescription and risk of ER, while those prescribed amiodarone or dronedarone in the BP were more likely to experience LR than those prescribed no AAD, which may represent an association due to confounding by indication.
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Antiarrítmicos/administración & dosificación , Fibrilación Atrial/terapia , Ablación por Catéter , Prescripciones de Medicamentos , Sistema de Registros , Anciano , Fibrilación Atrial/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , RecurrenciaRESUMEN
BACKGROUND: Studies have suggested that a fasciculoventricular pathway (FVP) may be the cause of preexcitation in patients with Danon disease, a rare X-linked dominant genetic disorder of hypertrophic cardiomyopathy. OBJECTIVE: The purpose of this study was to describe the prevalence of ventricular preexcitation on resting 12-lead electrocardiogram (ECG) in patients with Danon disease and the electrophysiological study (EPS) results of those with preexcitation. METHODS: Patients with confirmed Danon disease diagnosed with preexcitation (PR ≤120 ms, delta wave, QRS >110 ms) on ECG were included from a multicenter registry. The incidence of arrhythmias, implantable cardioverter-defibrillator (ICD) procedures, ICD shocks, and EPS results were collected. RESULTS: Thirteen of 40 patients (32.5%) with Danon disease were found to have preexcitation (mean age 17.3 years; 38% women). EPS performed in 9 of 13 patients (69%) demonstrated FVP only in 2 (22.2%), extranodal pathway without exclusion of FVP in 2 (22.2%), and both FVP and extranodal pathway in 5 (55.6%). Two patients had malignant accessory pathway (AP) properties. Over median follow-up of 842 days (interquartile range 138-1678), 11 patients (85%) had ICD placement, and 6 (46.1%) underwent heart transplantation. No patients required therapy for ventricular tachycardia, and 2 patients (15%) had paroxysmal atrial fibrillation. CONCLUSION: In a large multicenter cohort of patients with Danon disease, there was a high prevalence of FVP and extranodal pathways diagnosed on EPS in those with preexcitation. These findings suggest patients with preexcitation and Danon disease should undergo EPS to assess for FVP and potentially malignant extranodal AP.