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. 2021 Jul 29;21(6):349–366. doi: 10.1016/j.ipej.2021.07.006
COR Recommendations
LOE References
Neuromuscular Diseases and Other Progressive Cardiac Conduction Diseases
I Permanent pacemaker implantation is indicated in patients with neuromuscular diseases with symptomatic bradycardia due to SND or any degree of AV block. B-NR [2,31]
I Permanent pacemaker implantation is indicated in Kearns-Sayre syndrome for any degree of AV block (including first-degree AV block) and/or conduction abnormality because of unpredictable progression of conduction disease. C-LD [32]
IIa Permanent pacemaker implantation is reasonable in patients with myotonic dystrophy type 1 for marked first-degree AV block (PR interval >240 ms) or intraventricular conduction delay (native QRS duration >120 ms). Additional defibrillator capability may be considered. B-NR [33]
IIa Permanent pacemaker implantation is reasonable in patients with lamin A/C gene mutations, including limb-girdle and Emery-Dreifuss muscular dystrophies with a PR interval >240 ms and/or left bundle branch block. Additional defibrillator capability may be considered. C-LD [34]
IIb Permanent pacemaker implantation may be considered for any patient with any progressive cardiac conduction disease with potential for rapid deterioration of AV nodal function, even in the presence of normal AV conduction after taking into consideration patient age, size, and other individual risk factors. C-LD

Conditions include Duchenne muscular dystrophy, Becker muscular dystrophy, myotonic dystrophy type 1, Friedreich ataxia, Emery-Dreifuss muscular dystrophy, facioscapulohumeral muscular dystrophy, Barth syndrome, Kearns-Sayre syndrome, lamin A/C mutations, and desmin-related myopathies.