Risk of arrhythmias in MYotonic dystrophy: Trial design of the RAMYD study

Antonio Dello Russo, Fortunato Mangiola, Paolo Della Bella, Giovanni Nigro, Paola Melacini, Maria Grazia Bongiorni, Claudio Tondo, Leonardo Calò, Loredana Messano, Manuela Pace, Gemma Pelargonio, Michela Casella, Tommaso Sanna, Gabriella Silvestri, Anna Modoni, Elisabetta Zachara, Massimo Moltrasio, Lucia Morandi, Gerardo Nigro, Luisa PolitanoAlberto Palladino, Fulvio Bellocci

Research output: Contribution to journalArticle

Abstract

OBJECTIVE: Myotonic dystrophy type 1 (DM1) is the most frequent muscular dystrophy in adults. DM1 is a multisystem disorder also affecting the heart with an increased incidence of sudden death, which has been explained with the common impairment of the conduction system often requiring pacemaker implantation; however, the occurrence of sudden death despite pacemaker implantation and the observation of major ventricular arrhythmias generated the hypothesis that ventricular arrhythmias may play a causal role as well. The aim of the study was to assess the 2-year cumulative incidence and the value of noninvasive and invasive findings as predictive factors for sudden death, resuscitated cardiac arrest, ventricular fibrillation, sustained ventricular tachycardia and severe sinus dysfunction or high-degree atrioventricular block. METHODS/DESIGN: More than 500 DM1 patients will be evaluated at baseline with a clinical interview, 12-lead ECG, 24-h ECG and echocardiogram. Conventional and nonconventional indications to electrophysiological study, pacemaker, implantable cardioverter defibrillator or loop recorder implantation have been developed. In the case of an indication to electrophysiological study, pacemaker, implantable cardioverter defibrillator or loop recorder implant at baseline or at follow-up, the patient will be referred for the procedure. At the end of 2-year follow-up, all candidate prognostic factors will be tested for their association with the endpoints. Trial registration: ClinicalTrials.gov ID NCT00127582. CONCLUSION: The available evidence supports the hypothesis that both bradyarrhythmias and tachyarrhythmias may cause sudden death in DM1, but the course of cardiac disease in DM1 is still unclear. We expect that this large, prospective, multicenter study will provide evidence to improve diagnostic and therapeutic strategies in DM1.

Original languageEnglish
Pages (from-to)51-58
Number of pages8
JournalJournal of Cardiovascular Medicine
Volume10
Issue number1
DOIs
Publication statusPublished - Jan 2009

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Keywords

  • Arrhythmias
  • Electrophysiological study
  • Myotonic dystrophy type 1
  • Sudden death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Russo, A. D., Mangiola, F., Bella, P. D., Nigro, G., Melacini, P., Bongiorni, M. G., Tondo, C., Calò, L., Messano, L., Pace, M., Pelargonio, G., Casella, M., Sanna, T., Silvestri, G., Modoni, A., Zachara, E., Moltrasio, M., Morandi, L., Nigro, G., ... Bellocci, F. (2009). Risk of arrhythmias in MYotonic dystrophy: Trial design of the RAMYD study. Journal of Cardiovascular Medicine, 10(1), 51-58. https://doi.org/10.2459/JCM.0b013e328319bd2c