title: Risk of arrhythmias in myotonic dystrophy: trial design of the RAMYD study.
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
  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.
  
  
  BACKGROUND
  ClinicalTrials.gov ID NCT00127582.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University, L.go A. Gemelli 8, 00168 Rome, Italy. adellorusso@tin.it
authors.forename: Antonio
authors.initials: A
authors.lastname: Dello Russo
authors.forename: Fortunato
authors.initials: F
authors.lastname: Mangiola
authors.forename: Paolo
authors.initials: P
authors.lastname: Della Bella
authors.forename: Giovanni
authors.initials: G
authors.lastname: Nigro
authors.forename: Paola
authors.initials: P
authors.lastname: Melacini
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Bongiorni
authors.forename: Claudio
authors.initials: C
authors.lastname: Tondo
authors.forename: Leonardo
authors.initials: L
authors.lastname: Calò
authors.forename: Loredana
authors.initials: L
authors.lastname: Messano
authors.forename: Manuela
authors.initials: M
authors.lastname: Pace
authors.forename: Gemma
authors.initials: G
authors.lastname: Pelargonio
authors.forename: Michela
authors.initials: M
authors.lastname: Casella
authors.forename: Tommaso
authors.initials: T
authors.lastname: Sanna
authors.forename: Gabriella
authors.initials: G
authors.lastname: Silvestri
authors.forename: Anna
authors.initials: A
authors.lastname: Modoni
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Zachara
authors.forename: Massimo
authors.initials: M
authors.lastname: Moltrasio
authors.forename: Lucia
authors.initials: L
authors.lastname: Morandi
authors.forename: Gerardo
authors.initials: G
authors.lastname: Nigro
authors.forename: Luisa
authors.initials: L
authors.lastname: Politano
authors.forename: Alberto
authors.initials: A
authors.lastname: Palladino
authors.forename: Fulvio
authors.initials: F
authors.lastname: Bellocci
mesh_terms: D001145:Arrhythmias, Cardiac / Q000175:diagnosis / Q000209:etiology* / Q000517:prevention & control; D054537:Atrioventricular Block / Q000209:etiology; D002304:Cardiac Pacing, Artificial; D016887:Cardiopulmonary Resuscitation; D016757:Death, Sudden, Cardiac / Q000209:etiology; D017147:Defibrillators, Implantable; D018450:Disease Progression; D018572:Disease-Free Survival; D004452:Echocardiography; D004562:Electrocardiography; D022062:Electrophysiologic Techniques, Cardiac; D005260:Female; D006323:Heart Arrest / Q000209:etiology / Q000628:therapy; D006801:Humans; D007558:Italy; D053208:Kaplan-Meier Estimate; D008297:Male; D009223:Myotonic Dystrophy / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D010138:Pacemaker, Artificial; D011237:Predictive Value of Tests; D011446:Prospective Studies; D012107:Research Design; D018570:Risk Assessment; D017180:Tachycardia, Ventricular / Q000209:etiology; D013997:Time Factors; D014693:Ventricular Fibrillation / Q000209:etiology