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Journal of Neurology

, Volume 265, Issue 4, pp 885–895 | Cite as

A 34-year longitudinal study on long-term cardiac outcomes in DM1 patients with normal ECG at baseline at an Italian clinical centre

  • Elisabetta Bucci
  • Marco Testa
  • Loretta Licchelli
  • Alessandra Frattari
  • Nadia Attalla El Halabieh
  • Erica Gabriele
  • Giulia Pignatelli
  • Tiziana De Santis
  • Laura Fionda
  • Fiammetta Vanoli
  • Stefania Morino
  • Matteo Garibaldi
  • Antonella Di Pasquale
  • Nicola Vanacore
  • Annalisa Botta
  • Giovanni Antonini
Original Communication

Abstract

Cardiac conduction and/or rhythm abnormalities (CCRA) are the most frequent and life-threatening complications in DM1. In order to determine prevalence, incidence, characteristics, age of onset and predictors of CCRA, CCRA progression and sudden cardiac death (SCD) in DM1, we collected ECG/24hECG-Holter data from a yearly updated 34-year database of a cohort of 103 DM1 patients without cardiac abnormalities at baseline, followed for at least 1 year. Fifty-five patients developed CCRA [39 developed conduction abnormalities (CCA) and 16 rhythm abnormalities (CRA)], which progressed in 22. Nine had SCD. 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; risk and incidence of SCD amounted to 8.74 and 0.67% person-years, respectively. CTG expansion represented 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). MIRS progression during follow-up was associated 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). Age of CCRA onset and CCRA progression was significantly lower in patients with larger CTG expansion and in those with MIRS progression. Age when SCD occurred was significantly lower in patients with larger CTG expansion. Amongst recorded cardiac abnormalities, both atrial flutter (OR 8.70; p = 0.031) and paroxysmal supraventricular tachycardia (OR 8.67; p = 0.040) were associated with SCD. Although all DM1patients may develop cardiac abnormalities at any time in their life, patients older than 30 years with larger CTG expansion and MIRS progression in particular should be carefully monitored via periodical ECG.

Keywords

Myotonic dystrophy type 1 CTG expansion Cardiac conduction and/or rhythm abnormalities Sudden cardiac death Prevalence Incidence Risk factors 

Notes

Compliance with ethical standards

Conflicts of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

Ethical standard

This study has been approved by our Institution’s Ethics Committee and has, therefore, been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.

Supplementary material

415_2018_8773_MOESM1_ESM.pdf (92 kb)
Supplementary material 1 (PDF 93 kb)

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Elisabetta Bucci
    • 1
  • Marco Testa
    • 2
  • Loretta Licchelli
    • 1
  • Alessandra Frattari
    • 2
  • Nadia Attalla El Halabieh
    • 2
  • Erica Gabriele
    • 2
  • Giulia Pignatelli
    • 2
  • Tiziana De Santis
    • 1
  • Laura Fionda
    • 1
  • Fiammetta Vanoli
    • 1
  • Stefania Morino
    • 1
  • Matteo Garibaldi
    • 1
  • Antonella Di Pasquale
    • 1
  • Nicola Vanacore
    • 3
  • Annalisa Botta
    • 4
  • Giovanni Antonini
    • 1
  1. 1.Department of Neuroscience, Mental Health and Sensory Organs (NESMOS), School of Medicine and PsychologySapienza University of Rome, Sant’Andrea HospitalRomeItaly
  2. 2.Department of Cardiology, School of Medicine and PsychologySapienza University of Rome, Sant’Andrea HospitalRomeItaly
  3. 3.National Centre for Disease Prevention and Health PromotionNational Institute of HealthRomeItaly
  4. 4.Medical Genetic Section, Department of Biomedicine and PreventionTor Vergata UniversityRomeItaly

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