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Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

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22 Table 4. Clinical scenarios for the management of arrhythmias in DM1 Clinical scenario BMD 3. A 72-year-old woman with DM1 and significant skeletal muscle weakness presents with recurrent dizziness and falling over the past year. ECG shows sinus rhythm, PR interval 260 ms, and LBBB (QRS 160 ms). Echocardiogram shows LVEF 52% without wall motion abnormality. The 30-day event recorder shows sinus rhythm, occasional multiform premature ventricular complexes, and no symptomatic episodes. 4. A 68-year-old woman with DM1 and advanced neuromuscular impairment resulting in repeated falls is found to have asymptomatic rate-controlled AF during hospitalization for trochanteric fracture. She is expected to be confined to bed indefinitely. Other comorbidities include diabetes and chronic kidney disease stage 2. She has never had stroke/thromboembolism or serious bleeding. Ventricular function is normal; she has intact cognition and reports favorable quality of life with frequent family visitation. Scenarios cover different degrees of muscle impairment. (cont'd) Myotonic Dystrophy Types 1 and 2

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