Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders
Publication Date: April 27, 2022
Last Updated: May 8, 2023
Duchenne, Becker, and Recessive Limb-girdle Muscular Dystrophies
Diagnostic testing and risk stratification in Duchenne, Becker, and recessive limb-girdle muscular dystrophies
Coordinated care of patients with DMD, BMD, or LGMD2 should be conducted in a medical setting where there is access to expertise in the neurological, cardiac, arrhythmic, pulmonary, and genetic manifestations of these disorders. (I, B-NR)
573
In patients with DMD, BMD, or LGMD2, guideline-directed evaluation and therapy for heart failure is recommended. (I, B-NR)
573
In patients with DMD, BMD, or LGMD2, cardiac evaluation including physical examination, electrocardiogram (ECG), ambulatory ECG, and cardiac imaging (echocardiography or cardiac magnetic resonance imaging [CMR]) at diagnosis with periodic retesting is recommended even in the absence of cardiac symptoms. (I, B-NR)
573
In females who are carriers of a pathogenic or likely pathogenic variant for DMD or BMD, screening cardiac imaging (echocardiography or CMR) is recommended in adulthood even in the absence of cardiac symptoms. (I, B-NR)
573
In patients with DMD, BMD, or LGMD2 who have symptoms of conduction disorder or arrhythmias without an obvious cause, implantable cardiac monitoring is reasonable. (IIa, C-LD)
573
Bradycardias, conduction disorders, and use of pacing or CRT in Duchenne, Becker, and recessive limb-girdle muscular dystrophies
In patients with DMD, BMD, or LGMD2, with documented symptomatic bradycardia due to any degree of sinus node dysfunction or AV block, permanent pacemaker (PPM) implantation is indicated if concordant with the patient's goals of care and clinical status. (I, B-NR)
573
In patients with DMD, BMD, or LGMD2 and third-degree or advanced second-degree AV block at any anatomical level, with or without symptoms, PPM implantation is indicated if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with DMD, BMD, or LGMD2 with an LVEF ≤35% despite guideline-directed medical therapy (GDMT) with a combination of sinus rhythm, left bundle branch block (LBBB), QRS duration ≥150 ms, and New York Heart Association (NYHA) class II to class IV symptoms, or in those with suspected RV pacing-induced CM or anticipated RV pacing ≥40%, CRT is reasonable if concordant with the patient’s goals of care and clinical status. (IIa, B-NR)
573
Atrial arrhythmias in Duchenne, Becker, and recessive limb-girdle muscular dystrophies
In patients with DMD, BMD, or LGMD2, anticoagulation according to established guidelines and clinical context is recommended for AF or atrial flutter (AFL) taking into consideration the risks of thromboembolism and bleeding on oral anticoagulation. (I, B-NR)
573
VAs, sudden cardiac death, and use of ICDs in Duchenne, Becker, and recessive limb-girdle muscular dystrophies
In patients with DMD, BMD, or LGMD2 with spontaneously occurring hemodynamically significant sustained ventricular tachycardia (VT) or ventricular fibrillation (VF) ICD therapy is indicated if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with DMD, BMD, or LGMD2 with an LVEF ≤35% despite GDMT, ICD therapy is reasonable if concordant with the patient’s goals of care and clinical status. (IIa, B-NR)
573
Myotonic Dystrophy Types 1 and 2
Diagnostic testing and risk stratification in DM1 and DM2
Coordinated care of patients with DM1 or DM2 should be conducted in a medical setting where there is access to expertise in the neurological, cardiac, arrhythmic, pulmonary, and genetic manifestations of these disorders. (I, C-EO)
573
In patients with DM1 or DM2, cardiac evaluation including physical examination, ECG, ambulatory ECG, and cardiac imaging (echocardiography or CMR) at diagnosis with periodic retesting is recommended even in the absence of cardiac symptoms. (I, B-NR)
573
In patients with DM1 or DM2 and cardiac conduction disorder, close monitoring for arrhythmic complications is recommended when using mexiletine (or other sodium channel blockers). (I, C-LD)
573
In patients with DM1 or DM2 with symptoms consistent with bradycardia and with ECG evidence of mild to moderate conduction disorder and when noninvasive testing is nondiagnostic, electrophysiological (EP) testing is reasonable for risk stratification for AV block and sudden cardiac death. (IIa, B-NR)
573
In patients with DM1 or DM2 with symptoms suggestive of ventricular tachyarrhythmias and when noninvasive testing is nondiagnostic, EP testing to assess the risk of sustained arrhythmias may be considered. (IIb, B-NR)
573
Bradycardias, conduction disorders, and use of pacing or CRT in DM1 and DM2
In patients with DM1 or DM2 with an LVEF ≤35%, sinus rhythm, LBBB with a QRS duration ≥150 ms, and NYHA class II to class IV symptoms, or suspected RV pacing-induced CM despite GDMT, CRT is recommended if concordant with the patient’s goals of care and clinical status. (I, B-R)
573
In patients with DM1 or DM2 and documented symptomatic bradycardia due to any degree of sinus node dysfunction or AV block, PPM implantation is indicated if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with DM1 or DM2 and third-degree or advanced second-degree AV block at any anatomical level, with or without symptoms, PPM implantation is indicated if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with DM1 or DM2 and marked first-degree AV block (PR interval ≥240 ms) or intraventricular conduction delay (native QRS duration ≥120 ms), PPM implantation is reasonable if concordant with the patient’s goals of care and clinical status. (IIa, B-NR)
573
In patients with DM1 or DM2 with HV interval ≥70 ms on EP study, PPM implantation is reasonable if concordant with the patient’s goals of care and clinical status. (IIa, B-NR)
573
Atrial arrhythmias in DM1 and DM2
In patients with DM1 or DM2, anticoagulation according to established guidelines and clinical context is recommended for AF or AFL taking into consideration the risks of thromboembolism and the risks of bleeding on oral anticoagulation. (I, B-NR)
573
VAs, sudden cardiac death, and use of ICDs in DM1 and DM2
In patients with DM1 or DM2 in whom ICD therapy is planned, an ICD system with permanent pacing capability is recommended. (I, B-NR)
573
In patients with DM1 or DM2, who are survivors of spontaneously occurring hemodynamically significant sustained VT or VF, ICD therapy is indicated if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with DM1 or DM2 and an LVEF ≤35%, despite GDMT, ICD therapy is indicated if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with DM1 or DM2 in whom clinically relevant VAs are induced during EP study, ICD therapy is recommended if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with DM1 or DM2 in whom PPM implantation is indicated, ICD therapy may be considered if concordant with the patient’s goals of care and clinical status. (IIb, B-NR)
573
Emery-Dreifuss and Limb-girdle Type 1B Muscular Dystrophy
Diagnostic testing and risk stratification in EDMD and LGMD1B
Coordinated care of patients with EDMD or LGMD1B should be conducted in a medical setting where there is access to expertise in the neurological, cardiac, arrhythmic, pulmonary, and genetic manifestations of these disorders. (I, C-EO)
573
In patients with EDMD or LGMD1B, cardiac evaluation including physical examination, ECG, ambulatory ECG, and cardiac imaging (echocardiography or CMR) at diagnosis with periodic retesting is recommended even in the absence of cardiac symptoms. (I, B-NR)
573
First-degree relatives of patients with genetically confirmed EDMD or LGMD1B, who do not have access or have opted out of genetic testing, should be screened with ECG and cardiac imaging (echocardiography or CMR). (I, B-NR)
573
In patients with EDMD or LGMD1B, who have symptoms of conduction disorder or arrhythmias, implantable cardiac monitoring is reasonable, even in the setting of a normal 12-lead ECG, normal ambulatory ECG monitoring, and/or normal transthoracic echocardiogram. (IIa, C-EO)
573
In patients with EDMD or LGMD1B with symptoms consistent with bradycardia and ECG evidence of mild to moderate conduction disorder, or symptoms consistent with ventricular tachyarrhythmias, and when noninvasive testing is nondiagnostic, EP testing may be considered for risk stratification for sustained arrhythmias, AV block, and sudden cardiac death. (IIb, C-LD)
573
Bradycardias, conduction disorders, and use of pacing or CRT in EDMD and LGMD1B
In patients with EDMD or LGMD1B with an LVEF ≤35% despite GDMT, with a combination of sinus rhythm, LBBB, QRS duration ≥150 ms, and NYHA class II to class IV symptoms, or in those with suspected RV pacing-induced CM or anticipated RV pacing ≥40%, CRT is recommended if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with EDMD or LGMD1B in whom pacing is indicated and ICD therapy is not concordant with the patient’s goals of care and clinical status, a PPM or, if appropriate, CRT-P implantation is recommended. (I, C-EO)
573
Atrial arrhythmias in EDMD and LGMD1B
In patients with EDMD or LGMD1B, anticoagulation is recommended for AF or AFL, taking into consideration the risk of bleeding on oral anticoagulation. (I, B-NR)
573
In patients with EDMD, anticoagulation is recommended for atrial standstill, taking into consideration the risk of bleeding on oral anticoagulation. (I, B-NR)
573
VAs, sudden cardiac death, and use of ICDs in EDMD and LGMD1B
In patients with EDMD or LGMD1B in whom ICD therapy is planned, an ICD system with permanent pacing capability is recommended. (I, B-NR)
573
In patients with EDMD or LGMD1B who are survivors of spontaneously occurring hemodynamically significant sustained VT or VF, ICD therapy is indicated if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with EDMD or LGMD1B with at least one of the following: second-degree or third-degree AV block, PR interval ≥230 ms, or spontaneous HV ≥70 ms, ICD therapy is recommended if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with EDMD or LGMD1B with an LVEF ≤35% despite GDMT, ICD therapy is indicated if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with EDMD or LGMD1B in whom clinically relevant VAs are induced during EP study, ICD therapy is recommended if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with EDMD or LGMD1B with LVEF <45% and nonsustained VT, an ICD is reasonable if concordant with the patient’s goals of care and clinical status. (IIa, B-NR)
573
In patients with EDMD or LGMD1B with at least one of the following: LBBB, RBBB, or AF/AFL with slow ventricular response (ventricular rate <50 bpm), ICD therapy is reasonable if concordant with the patient’s goals of care and clinical status. (IIa, C-LD)
573
In patients with EDMD or LGMD1B with symptomatic sinus node dysfunction or sinus bradycardia with heart rate <40 bpm, ICD therapy may be considered if concordant with the patient’s goals of care and clinical status. (IIb, C-LD)
573
Facioscapulohumeral Muscular Dystrophy
Diagnostic testing and risk stratification in FSHD
In patients with FSHD, cardiac evaluation including examination, ECG, ambulatory ECG, and cardiac imaging (echocardiography or CMR) at diagnosis with periodic retesting are reasonable even in the absence of cardiac symptoms. (IIa, B-NR)
573
Mitochondrial Myopathies Including Friedreich Ataxia
Diagnostic testing and risk stratification in mitochondrial myopathies including FA
Coordinated care of patients with mitochondrial myopathies including FA should be conducted in a medical setting where there is access to expertise in the neurological, cardiac, arrhythmic, pulmonary, and genetic manifestations of these disorders. (I, B-NR)
573
In patients with mitochondrial myopathies including FA, cardiac evaluation including examination, ECG, ambulatory ECG, and cardiac imaging (echocardiography or CMR) at diagnosis with periodic retesting is recommended even in the absence of cardiac symptoms. (I, B-NR)
573
Bradycardias, conduction disorders, and use of pacing or CRT in mitochondrial myopathies including FA
In patients with mitochondrial myopathies including FA and documented symptomatic bradycardia due to sinus node dysfunction or any degree of AV block, PPM implantation is indicated if concordant with the patient's goals of care and clinical status. (I, B-NR)
573
In patients with mitochondrial myopathies including FA and third-degree or advanced second-degree AV block at any anatomical level, with or without symptoms, PPM implantation is indicated if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with FA with an LVEF ≤35% despite GDMT, with a combination of sinus rhythm, LBBB, QRS duration ≥150 ms, and NYHA class II to class IV symptoms, or in those with suspected RV pacing-induced CM or anticipated RV pacing ≥40%, CRT is reasonable if concordant with the patient’s goals of care and clinical status. (IIa, B-NR)
573
In patients with mitochondrial myopathies including FA with progressive ECG conduction disorder including any degree of AV or fascicular block, PPM implantation is reasonable if concordant with the patient’s goals of care and clinical status. (IIa, B-NR)
573
Atrial arrhythmias in mitochondrial myopathies including FA
In patients with mitochondrial myopathies including FA, anticoagulation according to established guidelines and clinical context is recommended for AF or AFL taking into consideration the risks of thromboembolism and the risks of bleeding on oral anticoagulation. (I, B-NR)
573
VAs, sudden cardiac death, and use of ICDs in mitochondrial myopathies including FA
In patients with mitochondrial myopathies including FA with spontaneously occurring VF or sustained hemodynamically significant VT, ICD therapy is indicated if concordant with the patient’s goals of care and clinical status. (I, B-NR)
573
In patients with mitochondrial myopathies including FA with an LVEF ≤35% despite GDMT, ICD therapy is reasonable if concordant with the patient’s goals of care and clinical status. (IIa, B-NR)
573
Shared Decision-making and End-of-life Care
Shared decision-making and end-of-life decisions
In patients with NMD who are considering or have a pacemaker or ICD, education on function including deactivation should be periodically discussed with the patient, their family members, and/or healthcare decision makers. (I, C-EO)
573
In patients with NMD in whom the presence of conduction disorder portends a risk of VAs, the decision of whether to implant a pacemaker or ICD should be concordant with the patient’s overall medical care goals and clinical status. (I, C-EO)
573
In patients with NMD who are considering ICD replacement and are undertaking advanced care planning, discussing the options of deferring ICD replacement is recommended. (I, C-EO)
573
In patients with NMD who have an ICD and are undertaking advanced care planning, discussing the option of deactivation of ICD shock therapy is recommended. (I, C-EO)
573
In patients with NMD who have an ICD and are experiencing VAs with shocks refractory to available therapies, discussion of management of ICD therapy including shock deactivation is recommended with careful attention to the patient’s goals of care. (I, C-EO)
573
In patients with NMD who have a pacemaker or ICD and who are nearing the end of life, if the patient or their healthcare decision maker requests pacing inactivation, it is reasonable to comply after education on the consequences of inactivation with careful attention to the patient’s goals of care. (IIa, C-EO)
573
Recommendation Grading
Disclaimer
The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.
Overview
Title
Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders
Authoring Organization
Heart Rhythm Society
Publication Month/Year
April 27, 2022
Last Updated Month/Year
October 3, 2024
Document Type
Consensus
Country of Publication
US
Document Objectives
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).
Target Patient Population
Patients with arrhythmic complications of neuromuscular disorders (NMDs)
Target Provider Population
Electrophysiologists, cardiologists, other clinicians, and health care professionals
Inclusion Criteria
Male, Female, Adolescent, Adult, Child, Older adult
Health Care Settings
Ambulatory, Hospital, Outpatient
Intended Users
Nurse, nurse practitioner, physician, physician assistant
Scope
Diagnosis, Assessment and screening, Management, Prevention
Diseases/Conditions (MeSH)
D001145 - Arrhythmias, Cardiac, D009468 - Neuromuscular Diseases
Keywords
arrhythmogenic cardiomyopathy, genetic arrhythmias, neuromuscular disorders, arrhythmic risk
Source Citation
Groh WJ, Bhakta D, Tomaselli GF, Aleong RG, Teixeira RA, Amato A, Asirvatham SJ, Cha YM, Corrado D, Duboc D, Goldberger ZD, Horie M, Hornyak JE, Jefferies JL, Kääb S, Kalman JM, Kertesz NJ, Lakdawala NK, Lambiase PD, Lubitz SA, McMillan HJ, McNally EM, Milone M, Namboodiri N, Nazarian S, Patton KK, Russo V, Sacher F, Santangeli P, Shen WK, Sobral Filho DC, Stambler BS, Stöllberger C, Wahbi K, Wehrens XHT, Weiner MM, Wheeler MT, Zeppenfeld K. 2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders. Heart Rhythm. 2022 Apr 26:S1547-5271(22)01946-4. doi: 10.1016/j.hrthm.2022.04.022. Epub ahead of print. PMID: 35500790.