Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

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)

In patients with DMD, BMD, or LGMD2, guideline-directed evaluation and therapy for heart failure is recommended. (I, B-NR)

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)

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)

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)

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)

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)

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)

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)

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)