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Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

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13 Management strategies Key points • Management options considered included primary prevention ICD implantation. • Continued cardiovascular medical therapy was recommended regardless of arrhythmia management strateg y. • Values elicited in discussion included current quality of life, actual anticipated benefit of ICD implantation, and expected surgical risks and recovery. • Deferral of ICD implantation was ultimately recommended and preferred due to unfavorable risk–benefit of device insertion. • Limited quality of life was raised as the main driver of the final management decision. • Lack of representation of patient substrate in previously published trials was noted. • Likely nonarrhythmic mechanism of death limits the benefit of ICD implantation. • Technical/procedural aspects of ICD implantation—kyphoscoliosis, sedation-related risks including respiratory infection, and possible prolonged recovery—were cited as additional determinants to defer ICD implantation. • Management options discussed included continued medical therapy, implantation of CRT-P, or implantation of CRT-D. • Values elicited in discussion included options to improve quality of life through treatment of heart failure symptoms and desire for protection against VAs. • CRT-D implantation was successfully performed to address the above issues. • CRT-D was indicated based on traditional guideline-based criteria. • Risk of sudden death due to VT or VF and ventricular dyssynchrony due to LBBB was addressed by CRT-D implantation. Relatively mild neuromuscular impairment, patient preference, and young age all lend themselves well to CRT-D (as opposed to CRT-P) implantation. • Following lengthy discussion, CRT-D was deemed compatible with patient's goals of care by all stakeholders.

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