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Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

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35 VAs, sudden cardiac death, and use of ICDs in mitochondrial myopathies including FA COR LOE Recommendations 1 B-NR 1. In patients with mitochondrial myopathies including FA with spontaneously occurring VF or sustained hemodynamically significant VT, ICD therapy is indicated if concordant with the patient's goals of care and clinical status. 2a B-NR 2. In patients with mitochondrial myopathies including FA with an LVEF ≤35% despite GDMT, ICD therapy is reasonable if concordant with the patient's goals of care and clinical status. Atrial arrhythmias in mitochondrial myopathies including FA COR LOE Recommendation 1 B-NR 1. In patients with mitochondrial myopathies including FA, 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. Bradycardias, conduction disorders, and use of pacing or CRT in mitochondrial myopathies including FA COR LOE Recommendations 2a B-NR 3. In patients with FA with an LVEF ≤35% despite GDMT, with a combination of sinus rhythm, LBBB, QRS duration ≥150 ms, and 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. 2a B-NR 4. In patients with mitochondrial myopathies including FA with progressive ECG conduction disorder including any degree of AV or fascicular block, PPM implantation is reasonable if concordant with the patient's goals of care and clinical status. (cont'd)

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