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Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

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12 Table 3. Clinical scenarios for the management of arrhythmias in DMD, BMD, and recessive LGMD2 Clinical scenario DMD 1. A 27-year-old man with DMD is found to have progressive left ventricular dysfunction (most recent LVEF 29%) despite maximally tolerated GDMT for >1 year. ECG shows sinus rhythm, PR interval 140 ms, QRS duration 100 ms, and a prominent R wave in lead V 1 . He requires a power wheelchair and full assistance for daily needs. Mechanical ventilation via tracheostomy is required, and nutrition is provided through enterostomy due to recurrent aspiration pneumonitis. Hospitalization for heart failure has not been observed. BMD 2. A 31-year-old man with BMD is found to have stable left ventricular dysfunction (most recent LVEF 32%) despite maximally tolerated GDMT for >1 year. He has exertional dyspnea and fatigue corresponding to NYHA function class III. He ambulates most of the day, uses a wheelchair for long distance mobility, and has a service animal to assist with activities of daily living. ECG shows sinus rhythm with LBBB with PR interval 160 ms, and QRS duration 150 ms. Duchenne, Becker, and Recessive Limb-girdle Muscular Dystrophies

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