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Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

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14 Duchenne, Becker, and Recessive Limb-girdle Muscular Dystrophies Table 3. Clinical scenarios for the management of arrhythmias in DMD, BMD, and recessive LGMD2 Clinical scenario LGMD2 3. A 38-year-old woman with LGMD2 reports several episodes of recurrent, unprovoked syncope, some resulting in minor injury. ECG shows sinus rhythm and right bundle branch block (RBBB). Echocardiogram shows normal LVEF with biatrial dilation. The 30-day event recorder shows sinus pauses with ventricular asystole (2.5–4.0 seconds) with overall heart rate range 60–110 beats per minute (bpm). She ambulates with assistance from her husband and the use of a rolling walker. (cont'd)

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