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Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

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17 Bradycardias, conduction disorders, and use of pacing or CRT in DM1 and DM2 COR LOE Recommendations 2a B-NR 4. In patients with DM1 or DM2 and marked first-degree AV block (PR interval ≥240 ms) or intraventricular conduction delay (native QRS duration ≥120 ms), PPM implantation is reasonable if concordant with the patient's goals of care and clinical status. 2a B-NR 5. In patients with DM1 or DM2 with HV interval ≥70 ms on EP study, PPM implantation is reasonable if concordant with the patient's goals of care and clinical status. Atrial arrhythmias in DM1 and DM2 COR LOE Recommendation 1 B-NR 1. In patients with DM1 or DM2, anticoagulation according to established guidelines and clinical context is recommended for AF or AFL taking into consideration the risks of thromboembolism and the risks of bleeding on oral anticoagulation. VAs, sudden cardiac death, and use of ICDs in DM1 and DM2 COR LOE Recommendations 1 B-NR 1. In patients with DM1 or DM2 in whom ICD therapy is planned, an ICD system with permanent pacing capability is recommended. 1 B-NR 2. In patients with DM1 or DM2, who are survivors of spontaneously occurring hemodynamically significant sustained VT or VF, ICD therapy is indicated if concordant with the patient's goals of care and clinical status. 1 B-NR 3. In patients with DM1 or DM2 and an LVEF ≤35%, despite GDMT, ICD therapy is indicated if concordant with the patient's goals of care and clinical status. 1 B-NR 4. In patients with DM1 or DM2 in whom clinically relevant VAs are induced during EP study, ICD therapy is recommended if concordant with the patient's goals of care and clinical status. 2b B-NR 5. In patients with DM1 or DM2 in whom PPM implantation is indicated, ICD therapy may be considered if concordant with the patient's goals of care and clinical status. (cont'd)

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