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Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

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23 Management strategies Key points • Management options discussed included use of further noninvasive and invasive diagnostic strategies versus preemptive arrhythmia therapies as follows: ▶ Prolonged arrhythmia monitoring with loop recorder insertion ▶ EP testing to assess AV conduction and evaluate the inducibility of VAs, followed by CIED insertion ▶ Empiric pacemaker implantation ▶ Empiric transvenous ICD implantation • Values elicited in discussion included desire to avoid complications from symptomatic episodes, most expeditious management strategy, and focus on quality of life. • Although several risk indicators for sudden death were present (PR interval 260 ms and LBBB), the most likely serious etiology for observed episodes remained bradyarrhythmias due to high-grade AV block. Advanced functional impairment and primary emphasis on quality of life led to the decision to pursue empiric pacemaker implantation. • Age, significant functional impairment, patient wishes, and suggestive clinical features were key points in determining ultimate management strateg y. • Transvenous ICD would accomplish protection against brady- and tachyarrhythmias, but limited evidence, possible longer adjustment (compared to pacemaker), and patient wishes favored pacemaker implantation. • Management options discussed included prescribing direct oral anticoagulant/ warfarin, left atrial appendage occlusion, and avoidance of anticoagulation altogether. • Values elicited in discussion included reduced though acceptable quality of life, desire to avoid preventable life-threatening/ life-altering medical complications, preference for noninvasive therapy, increased thromboembolic risk balanced by limited bleeding risk using conventional risk calculators, and limited fall risk given bed- confined status. • With CHA 2 DS 2 -VASc and HAS-BLED risk scores of 3 and 2, respectively, oral anticoagulation was recommended and accepted. • Conventional risk calculators are recommended for use in DM1 patients and support the use of oral anticoagulation here. • Fall risk and frailty are difficult to quantify but must be considered when considering anticoagulation. • Patient wishes to avoid serious preventable complications through noninvasive means were heeded; anticoagulation will reduce the risk of systemic thromboembolism due to AF.

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