COR LOE Recommendations I B-NR In patients with neuromuscular disorders, primary and secondary prevention ICDs are recommended for the same indications as for patients with NICM if meaningful survival of greater than 1 year is expected.S7.8-1,S7.8-2 IIa B-NR In patients with Emery-Dreifuss and limb- girdle type IB muscular dystrophies with progressive cardiac involvement, an ICD is reasonable if meaningful survival of greater than 1 year is expected.S7.8-3 S7.8-8 IIa B-NR In patients with muscular dystrophy, follow- up for development of cardiac involvement is reasonable, even if the patient is asymptomatic at presentation.S7.8-9 S7.8-12 IIb B-NR In patients with myotonic dystrophy type 1 with an indication for a permanent pacemaker, an ICD may be considered to minimize the risk of SCA from VT if meaningful survival of greater than 1 year is expected.S7.8-9,S7.8-13,S7.8-14 Synopsis The muscular dystrophies are a group of inherited diseas- es affecting skeletal and cardiac muscle. Some present primarily as a NICM (eg, Duchenne, Becker, and limb-gir- dle types 2C, 2F, and 2I), while others present primarily as conduction system degeneration with a variable associa- tion with cardiomyopathy (eg, myotonic dystrophy types 1 and 2, Emery-Dreifuss, limb-girdle type 1B; summa- rized in Table 9).S7.8-15 Because SCD can occur either due to VA or due to bradyarrhythmias from rapid and unpre- dictable progression of conduction system disease, the clinician is faced with the challenge of identifying those patients who would benefit from prophylactic pacemak- CLINICAL STATEMENTS AND GUIDELINES er or ICD implantation. There should be a high level of concern for those patients with muscular dystrophy who present with arrhythmia symptoms.S7.8-15 The current guideline focuses on VA and indications for implantation of an ICD. The indications for permanent pacemaker are discussed in another ACC/AHA/HRS guideline.S7.8-16 Recommendation-Specific Supportive Text 1. 