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Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

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43 Management strategies Key points • Acute management options included patient preference for enteral feeds, medical management of aspiration pneumonitis, and escalation of care if further deterioration was observed. • ICD management options discussed included unchanged programming and inactivation of tachyarrhythmia therapies with or without inactivation of pacing/CRT function. • Values elicited in discussion include decision for do-not-resuscitate status versus comfort measures with hospice referral. • Decision was made to continue nutrition via enterostomy, administering intravenous antibiotics with supplemental oxygen and inactivating tachyarrhythmia therapies, and maintaining pacing/ CRT programming. • Do-not-resuscitate status was requested in keeping with focus on quality of life. • Advanced neuromuscular impairment and associated medical conditions negatively impact quality of life. • Given poor overall prognosis, focus was shifted to maintaining quality over quantity of life. • Therapies targeting acute and possible reversible medical conditions were planned with avoidance of care escalation given unlikely benefit from aggressive measures and maintaining palliative therapies such as pacing/CRT. • Involvement of hospice medicine and palliative care specialists may be helpful in guiding and/or leading these discussions, with a focus on shared decision making. • Management options discussed included resumption of oral anticoagulation with warfarin or direct oral anticoagulant, left atrial appendage occlusion when appropriate, or observation without further intervention. • Values elicited in discussion included addressing preventable causes of morbidity and mortality and minimizing/avoidance of complications from medical/surgical thromboembolism prevention. • Patient's family declined resuming oral anticoagulation and deferred left atrial appendage indefinitely in favor of monitoring for further clinical neurological improvement given preference for conservative management. • Traditional appropriate management strategies may be fraught with an increased risk of complications in patients with neuromuscular conditions. • Invasive strategies may be poorly tolerated, associated with increased procedural risk, and less appropriate in patients with advanced neuromuscular impairment and associated complexities. • The benefit of commonly indicated therapies may be overshadowed by comorbidities related to the underlying condition.

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