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Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

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9 Atrial arrhythmias in Duchenne, Becker, and recessive limb- girdle muscular dystrophies COR LOE Recommendation 1 B-NR 1. 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. VAs, sudden cardiac death, and use of ICDs in Duchenne, Becker, and recessive limb-girdle muscular dystrophies COR LOE Recommendations 1 B-NR 1. 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. 2a B-NR 2. 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.

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