Heart rate turbulence as a noninvasive risk predictor of ventricular tachyarrhythmias in myotonic dystrophy type 1

Michela Casella, Antonio Dello Russo, Manuela Pace, Gemma Pelargonio, Carolina Ierardi, Tommaso Sanna, Loredana Messano, Gianluigi Bencardino, Sergio Valsecchi, Fortunato Mangiola, Gaetano A. Lanza, Paolo Zecchi, Filippo Crea, Fulvio Bellocci

Research output: Contribution to journalArticlepeer-review


Introduction: Myotonic dystrophy type 1 (MD1) is the most common muscular dystrophy of adult life. Cardiac involvement is characterized by disorders of atrioventricular conduction, ventricular arrhythmias, and sudden death. Heart rate turbulence (HRT) is a noninvasive risk predictor in patients affected by ischemic heart disease. The aim of our study is to assess the prognostic value of HRT in MD1 patients. Methods and Results: We performed HRT analysis by 24-hour Holter recording to calculate turbulence onset (TO) and turbulence slope (TS) in 29 MD1 patients (mean age 52 ± 10 years), and in 30 patients (mean age 52 ± 13 years) with frequent ventricular arrhythmias and structurally normal heart (VANH). An electrophysiological study (EPS) tested ventricular arrhythmias inducibility in 22 MD1 patients. TO was significantly different between MDl and VANH patients (-1.66 ± 2.04 and -2.98 ± 1.79%, respectively, P 0.01), while no difference was observed in TS between MD1 and VANH patients (11.12 ± 6.46 and 9.12 ± 6 msec/beat, respectively). On EPS, sustained ventricular arrhythmias (SVA) were induced in six MD1 patients. TO was significantly different in inducible MD1 patients (0.88 ± 1.95%), as compared with both noninducible (-2.49 ± 1.43%, P <0.001) or no eligible to EPS (-1.93 ± 1.63%, P <0.005) MD1 patients and to VANH patients (-2.98 ± 1.79%, P <0.001). Conclusions: An impairment of TO, a measure of HRT, suggesting impaired cardiac parasympathetic activity, may be a useful, noninvasive predictor of arrhythmic risk in MD1 patients.

Original languageEnglish
Pages (from-to)871-876
Number of pages6
JournalJournal of Cardiovascular Electrophysiology
Issue number8
Publication statusPublished - Aug 2006


  • Heart rate turbulence
  • Myotonic dystrophy
  • Ventricular arrhythmias

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology


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