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Competing Interests: Disclosures The authors have no conflicts of interest to disclose.

This international multidisciplinary document is intended to guide electrophysiologists, cardiologists, other clinicians, and health care professionals in caring for patients with arrhythmic complications of neuromuscular disorders (NMDs). The document presents an overview of arrhythmias in NMDs followed by detailed sections on specific disorders: Duchenne muscular dystrophy, Becker muscular dystrophy, and limb-girdle muscular dystrophy type 2; myotonic dystrophy type 1 and type 2; Emery-Dreifuss muscular dystrophy and limb-girdle muscular dystrophy type 1B; facioscapulohumeral muscular dystrophy; and mitochondrial myopathies, including Friedreich ataxia and Kearns-Sayre syndrome, with an emphasis on managing arrhythmic cardiac manifestations. End-of-life management of arrhythmias in patients with NMDs is also covered. The document sections were drafted by the writing committee members according to their area of expertise. The recommendations represent the consensus opinion of the expert writing group, graded by class of recommendation and level of evidence utilizing defined criteria. The recommendations were made available for public comment; the document underwent review by the Heart Rhythm Society Scientific and Clinical Documents Committee and external review and endorsement by the partner and collaborating societies. Changes were incorporated based on these reviews. By using a breadth of accumulated available evidence, the document is designed to provide practical and actionable clinical information and recommendations for the diagnosis and management of arrhythmias and thus improve the care of patients with NMDs., (Copyright © 2022 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: No comparative study of outcomes in Riata and Sprint Fidelis leads undergoing lead extraction (LE), lead abandonment (LA), and generator change only (GC) has been published., Objectives: Determine outcomes (major complications [MC]; death, extended hospitalization, or rehospitalization within 60 days [RH]; lead malfunction) of LE, LA, and GC for recalled leads., Methods: Retrospective, multicenter, comparative study., Results: A total of 298 LE, 85 LA, and 310 GC were performed. In the clinical setting of a lead intervention, there was no difference in a composite of MC, death, RH, lead revision, inappropriate shocks, or device infection between LE and LA groups (15% vs 22%, P = .140). In the clinical setting of a device at elective replacement interval (ERI), there were significantly more acute events at 60 days (MC, death, and RH) in the LE and LA groups at 15.4% (4) and 15.4% (4), and this was significantly (P = .017) higher than the GC group at 5.1% (16). There was no difference (P = 1.000) in the composite of MC, death, RH, lead malfunction, lead revisions, device infections, or inappropriate shocks between LE, LA, and GC groups at 15.4% (4), 15.4% (4), and 17.4% (54), respectively. Following generator change, 14 of 175 Fidelis leads and 3 of 135 Riata leads failed over a total of 12,714 months of follow-up., Conclusions: The failure rate of recalled leads was substantially lower compared to previous reports. It may be prudent to perform generator change only when the device is at ERI, especially when the recalled lead has historical performance that likely outweighs the risks of extraction/abandonment., (Copyright © 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: There is controversy whether proceduralist-directed, nurse-administered propofol sedation (PDNAPS) is safe., Objective: To assess the frequency of adverse events when PDNAPS is used for implantable cardioverter-defibrillator (ICD)-related procedures and to determine the patient and procedural characteristics associated with adverse events., Methods: Consecutive ICD-related procedures using PDNAPS from May 2006 to July 2009 at a tertiary-care hospital were evaluated. Serious adverse events were defined as procedural death, unexpected transfer to an intensive care unit, respiratory failure requiring intubation/bag-mask ventilation, or hypotension requiring vasoconstrictor/inotrope support. Nonserious adverse events were defined as hypotension requiring fluid resuscitation or hypoxemia requiring augmented respiratory support with non-rebreather mask, oral airway, or jaw lift., Results: Of 582 patients (age 64 ± 14 years, 72.3% males) undergoing ICD-related procedures using PDNAPS, 58 (10.0%) patients had serious adverse events with no procedural death and 225 (38.7%) had nonserious adverse events. Longer procedure duration (relative risk [RR] = 2.1 per hour; 95% confidence interval [CI] = 1.6-2.8; P < .001) and biventricular implant (RR = 2.7; CI = 1.4-5.3; P = .003) were independent predictors of serious adverse events. A longer procedure duration (RR = 1.4 per hour; CI = 1.1-1.7; P = .001), heart failure class (RR = 1.4 per 1 class; CI = 1.1-1.7; P = .002), and use of propofol infusion (RR = 3.5; CI = 2.2-5.7; P < .001) were independent predictors of nonserious adverse events., Conclusion: PDNAPS for shorter ICD procedures including single- and dual-chamber implants, generator changes, and defibrillation threshold testing have acceptable rates of serious adverse events and manageable nonserious adverse events and should be considered for further study. Biventricular implants and other complex procedures should be done with an anesthesiologist., (Copyright © 2012 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Cardiac tamponade (CT) is a possible complication of radiofrequency catheter ablation (RFCA) of atrial fibrillation (AF). Although the incidence of CT is not higher when RFCA is performed with a therapeutic international normalized ratio (INR), outcomes of CT are unclear., Objective: We compared outcomes among patients with and without a therapeutic INR who developed CT as a complication of RFCA of AF., Methods: The subjects of this retrospective study were 40 consecutive patients who developed CT during RFCA of AF at 3 centers. We divided the patients into 2 groups: RFCA performed with INR < 2 (group 1) and INR ≥ 2 (group 2). There were 23 patients in group 1 and 17 patients in group 2., Results: Baseline clinical and procedure characteristics were not different between the 2 groups. Heparin was reversed by protamine in 83% and 94% of patients (P = .37), and warfarin was reversed by fresh frozen plasma or factor VIIa in 17% and 35% of patients (P = .27) in groups 1 and 2, respectively. All patients were successfully treated by percutaneous drainage, and none required surgical intervention. There were no significant differences in the amount of initial pericardial drainage (523 ± 349 ml vs. 409 ± 157 ml, P = .22) or the duration of drainage (P = .14) between the 2 groups. All patients survived to hospital discharge. Median length of hospital stay was 2 days longer in group 1 (P <.01)., Conclusion: Cardiac tamponade is not more severe or difficult to manage in the presence of therapeutic anticoagulation with warfarin in patients undergoing RFCA of AF., (Published by Elsevier Inc.)

Background: Nonischemic dilated cardiomyopathy (NICM) is associated with diffuse global hypokinesia on echocardiography. However, NICM also may be associated with segmental wall-motion abnormalities (SWMAs) even in the presence of global hypokinesia, probably secondary to patchy myocardial scars., Objective: Because myocardial scars serve as substrate for reentry, the purpose of this study was to determine whether SWMA is a predictor of ventricular arrhythmic events in NICM., Methods: Echocardiographic parameters and appropriate implantable cardioverter-defibrillator (ICD) therapy for arrhythmic events (shock or antitachycardia pacing) were studied in NICM patients with an ICD. Two-dimensional echocardiography of the left ventricle was recorded in a 16-segment model. SWMA was defined by the presence of akinesia or moderate to severe hypokinesia in at least two segments. Patients were divided into one of two groups according to the presence (SWMA group) or the absence (non-SMWA group) of SWMA., Results: SWMA was present in 47.5% of 101 patients (mean age 58.0 ± 15.6 years, 85% male, primary prophylaxis indication 46%, mean ejection fraction 26% ± 9%, mean follow-up 29 ± 18.4 months) studied. No significant difference in mean age, ejection fraction, and QRS duration was seen between SWMA and non-SWMA groups. The SWMA group had a significantly higher incidence of arrhythmic events than did the non-SWMA group (65% vs 15%, P <.001). Kaplan-Meier survival analysis revealed that SMWA was associated with significantly reduced time to first arrhythmic event (P = .001). SWMA (P <0.001), New York Heart Association heart failure class (P = .016), and secondary prevention indication for ICD placement (P = .005) were significant independent predictors of an arrhythmic event. SWMA did not predict mortality., Conclusion: SWMA is an independent predictor of arrhythmic events in patients with NICM., (Copyright © 2010. Published by Elsevier Inc.)

Background: Myocardial scar is a substrate for reentrant ventricular arrhythmias and is associated with poor prognosis. Fragmented QRS (fQRS) on 12-lead ECG represents myocardial conduction delays due to myocardial scar in patients with coronary artery disease (CAD)., Objective: The purpose of this study was to determine whether fQRS is associated with increased ventricular arrhythmic event and mortality in patients with CAD and nonischemic dilated cardiomyopathy (DCM)., Methods: Arrhythmic events and mortality were studied in 361 patients (91% male, age 63.3 +/- 11.4 years, mean follow-up 16.6 +/- 10.2 months) with CAD and DCM who received an implantable cardioverter-defibrillator for primary or secondary prophylaxis. fQRS included various RSR' patterns (QRS duration <120 ms), such as > or =1 R prime or notching of the R wave or S wave present on at least two contiguous leads of those representing anterior (V(1)-V(5)), lateral (I, aVL, V(6)), or inferior (II, III, aVF) myocardial segments., Results: fQRS was present in 84 (23%) patients (fQRS group) and absent in 100 (28%) patients (non-fQRS group). Wide QRS (wQRS; QRS duration > or =120 ms) was present in 177 (49%) patients. Kaplan-Meier analysis revealed that event-free survival for an arrhythmic event (implantable cardioverter-defibrillator shock or antitachycardia pacing) was significantly lower in the fQRS group than in the non-fQRS and wQRS groups (P <.001 and P <.019, respectively). fQRS was an independent predictor of an arrhythmic event but not of death., Conclusion: fQRS on 12-lead ECG is a predictor of arrhythmic events in patients with CAD and DCM. fQRS is associated with a significantly decreased time to first arrhythmic event compared with non-fQRS and wQRS.