Bucci E, et al. - An inquiry was set up with regard to the prevalence, incidence, characteristics, age of onset and predictors of cardiac conduction and/or rhythm abnormalities (CCRA), CCRA progression and sudden cardiac death (SCD) in DM1 patients. It was discovered that all DM1 subjects could develop cardiac abnormalities at any time in their life. Nevertheless, cautious monitoring through periodical ECG was recommended particularly for individuals older than 30 years with larger CTG expansion and MIRS progression.

Methods

• Researchers extracted ECG/24hECG-Holter data from a yearly updated 34-year database of 103 DM1 patients without cardiac abnormalities at baseline, with at least 1 year follow-up.

Results

• It was noted that 55 subjects developed CCRA [39 developed conduction abnormalities (CCA) and 16 rhythm abnormalities (CRA)], which progressed in 22.
• A total of 9 patients had SCD.
• Data illustrated that risk and incidence of CCRA amounted to 53.4 and 6.83% person-years (CCA: 37.9 and 4.8%; CRA 15.5 and 2%), respectively.
• Additionally, the risk and incidence of SCD amounted to 8.74 and 0.67% person-years, respectively.
• As per the outcomes, CTG expansion served as a predictor of CCRA incidence (HR 1.10, p=0.04), CCRA progression (HR 1.28, p=0.001) and SCD (HR 1.39, p=0.002).
• A link was disclosed between MIRS progression during follow-up with CCRA prevalence (OR 5.82, p=0.004); older age and larger CTG expansion to SCD prevalence (OR 2.67, p=0.012; OR 1.54, p=0.005).
• A substantially lower age of CCRA onset and CCRA progression was reported in subjects with larger CTG expansion and among those with MIRS progression.
• It was discovered that age, when SCD occurred, appeared to be markedly lower in patients with larger CTG expansion.
• Atrial flutter (OR 8.70; p=0.031) and paroxysmal supraventricular tachycardia (OR 8.67; p=0.040) exhibited a correlation with SCD amongst recorded cardiac abnormalities.