![]() Basal surface ECG showed sinus rhythm with a PR interval of 0.16 seconds and a QRS duration of 0.10 seconds. On arrival at our department the vital signs were the following: blood pressure 110/70 mmHg, peripheral oxygen saturation 99% in room air, heart rate 70 bpm. ![]() The patient reported a case of SD in her family history (maternal grandmother, at age 29). ![]() Needle electromyography (EMG) showed mild myopathic changes and myotonic discharges. The patient showed temporal muscle atrophy, proximal weakness at lower limbs and grip and evoked myotonia. She was diagnosed with DM1 three years earlier showing an increased CTG repeat length, with a number size defined between 50-500. We report the case of a mildly symptomatic DM1 patient who underwent ICD implantation for high ventricular vulnerability at the electrophysiological evaluation even in the absence of either conduction disturbances at ECG or ventricular dysfunction/fibrosis at non-invasive evaluation by echocardiogram and cardiac magnetic resonance.Ī 42 year-old woman, affected by poorly symptomatic DM1 presented at our emergency department for syncope and palpitations. However, recent data showed that SD, striking up to one third of patients, can also occur for ventricular tachyarrhythmias (VTA) and that other predictive factors like syncope, family history of SD or non-sustained VT should be taken into account for risk stratification 4. Conduction system disturbances on surface ECG or ventricular dysfunction are considered the most relevant risk factors for sudden cardiac death (SD), favouring pacemaker (PM) implantation according to latest guidelines 2, 3. Cardiac involvement is reported in about 80% of cases, even in asymptomatic patients 1. Myotonic dystrophy type 1 (DM1) is the most frequent muscular dystrophy in adults.
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