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Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

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21 Management strategies Key points • Management options discussed included use of further noninvasive and invasive diagnostic strategies versus empiric arrhythmia therapies as follows: ▶ Prolonged ambulatory monitoring or loop recorder insertion ▶ Empiric pacemaker implantation given evidence of conduction system disease ▶ EP testing to assess AV conduction and evaluate the inducibility of VAs, followed by CIED insertion • Values elicited in discussion included likelihood of recurrence with further injuries, potential for life-threatening brady- and tachyarrhythmias as causative etiolog y, and favorable functional status with reasonable expected longevity. • Given age, high functional status, and potentially serious causative arrhythmias, EP testing was performed. Pacemaker implantation was planned with possible ICD insertion if clinically relevant VAs were induced. • Age, high functional status, and serious nature of syncope with injury prompted aggressive evaluation. • Empiric pacemaker implantation without further testing could be considered given existing RBBB. • Normal LVEF suggests absence of significant myocardial involvement. • Clinical benefit of empiric ICD implantation in this situation remains uncertain. • EP study was primarily utilized to determine the suitability of ICD implantation, as pacemaker implantation was appropriate with clinical features at presentation. • Management options discussed included implantation of dual-chamber pacemaker, biventricular pacemaker (CRT-P), and biventricular ICD (CRT-D) implantation. • Values elicited in discussion included desire for improved functional capacity, reduction of symptoms attributed to bradycardia and left ventricular dysfunction, and prevention of sudden death due to malignant brady- and tachyarrythmias. • The decision was made to proceed with biventricular ICD (CRT-D) implantation, as this would address all the relevant cardiovascular issues described. • Restoration of AV synchrony with alleviation of related symptoms was accomplished by permanent pacing. • Anticipated RV pacing ≥40% coupled with moderate preexisting left ventricular dysfunction warrants implantation of CRT device. • ICD implantation may be considered in DM1 patients who require pacing due to ongoing risk of sudden death, possibly due to VAs. • Moderate left ventricular dysfunction indicates myocardial involvement/infiltration due to DM1. • Beta-adrenergic blockade for left ventricular dysfunction may be safely used following device insertion without concern for aggravating bradycardia.

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