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Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

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46 Table 8. Clinical scenarios for end-of-life management in patients with NMD Clinical scenario 4. A 46-year-old woman with DM1 and severe neuromuscular impairment (nonambulatory, nocturnal oxygen supplementation) presents for first cardiac EP evaluation with referral from neurology service. No symptoms attributable to cardiac involvement or arrhythmias are noted. The ECG shows sinus rhythm, PR interval 260 ms, RBBB, and left anterior fascicular block with QRS duration 140 ms. Echocardiogram shows LVEF 55% without other significant abnormalities. Shared Decision-making and End-of-life Care (cont'd)

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