Evaluation of mexiletine effect on conduction delay and bradyarrhythmic complications in patients with myotonic dystrophy type 1 over long-term follow-up.

Heart Rhythm : the Official Journal of the Heart Rhythm Society
Riccardo VioChiara Calore

Abstract

Myotonic dystrophy type 1 (DM1) is a multisystemic disorder characterized by progressive cardiac conduction impairment, arrhythmias, and sudden death. Mexiletine is a sodium channel blocker drug used by patients with DM1 for treatment of myotonia, even though definitive proof of its safety over long-term follow-up is lacking. The purpose of this study was to assess the impact of mexiletine for treatment of neurological symptoms on the composite endpoint of significant electrocardiogram modification (new onset or worsening of atrioventricular [AV] or intraventricular conduction delay) and bradyarrhythmic complications requiring pacemaker (PM) implantation (advanced AV block, symptomatic sinus pause >3 seconds). This retrospective longitudinal study included a series of consecutive patients with genetically confirmed DM1 evaluated at our neurology and cardiology clinics from January 1, 2011, to January 1, 2020, who received mexiletine 200 mg twice daily. Patients with a PM, implantable cardioverter-defibrillator, or severe conduction abnormality (PQ interval ≥230 ms, complete bundle branch block, or atrial fibrillation) at enrollment were excluded. The study comprised 18 mexiletine-treated patients and 68 mexiletine-free controls...Continue Reading

Citations

Dec 22, 2020·Heart Rhythm : the Official Journal of the Heart Rhythm Society·Riccardo VioChiara Calore
Dec 22, 2020·Heart Rhythm : the Official Journal of the Heart Rhythm Society·Guillermo Gutiérrez-BallesterosJuan Jiménez-Jáimez
Apr 3, 2021·Drug Discovery Today·Marta Pascual-GilabertRuben Artero
Jun 15, 2021·Revista Española De Cardiología·Rafael Salguero-BodesFernando Arribas-Ynsaurriaga
May 15, 2021·Current Opinion in Neurology·Federica Montagnese, Benedikt Schoser

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