The frequency and severity of cardiac involvement in myotonic dystrophy type 2 (DM2): Long-term outcomes

V. A. Sansone, E. Brigonzi, B. Schoser, S. Villani, M. Gaeta, G. De Ambroggi, F. Bandera, L. De Ambroggi, G. Meola

Research output: Contribution to journalArticle


Background: Frequency and severity of cardiac involvement in DM2 are still controversial. The aims of our study were to determine the frequency and progression of cardiac and muscle involvement in a relatively large cohort of patients with DM2 throughout Italy and Germany and to provide long-term outcomes in this disorder. Methods: 104 DM2 and 117 DM1 patients underwent baseline and follow-up assessments of, ECG, 24 h Holter monitoring, 2D echocardiography and electrophysiological study (EPS) when appropriate, and manual muscle strength testing (mean follow-up: 7.4±4.1 for DM2 and 5.7±4 years for DM1). Results: Overall, 10% of DM2 patients vs 31% of DM1 patients had PR≥200 ms and 17% of DM2 patients vs 48% of DM1 patients had QRSD≥100 ms. Six patients with DM2 vs 28 patients with DM1 required PM/ICD implantations. DM2 patients were stronger than DM1 patients at baseline, but muscle strength worsened significantly over time (p

Original languageEnglish
Pages (from-to)1147-1153
Number of pages7
JournalInternational Journal of Cardiology
Issue number2
Publication statusPublished - Sep 30 2013


  • Cardiac arrhythmias
  • Myotonic dystrophy type 1
  • Myotonic dystrophy type 2
  • Sudden cardiac death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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