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Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

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8 Bradycardias, conduction disorders, and use of pacing or CRT in Duchenne, Becker, and recessive limb-girdle muscular dystrophies COR LOE Recommendations 1 B-NR 1. 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. 1 B-NR 2. 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. 2a B-NR 3. In patients with DMD, BMD, or LGMD2 with an LVEF ≤35% despite guideline-directed medical therapy (GDMT) with a combination of sinus rhythm, le 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. Duchenne, Becker, and Recessive Limb-girdle Muscular Dystrophies

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