title: Incidence and predictors of sudden death, major conduction defects and sustained ventricular tachyarrhythmias in 1388 patients with myotonic dystrophy type 1.
abstract: OBJECTIVE
  To describe the incidence and identify predictors of sudden death (SD), major conduction defects and sustained ventricular tachyarrhythmias (VTA) in myotonic dystrophy type 1 (DM1).
  
  
  RESULTS
  We retrospectively enrolled 1388 adults with DM1 referred to six French medical centres between January 2000 and October 2013. We confirmed their vital status, classified all deaths, and determined the incidence of major conduction defects requiring permanent pacing and sustained VTA. We searched for predictors of overall survival, SD, major conduction defects, and sustained VTA by Cox regression analysis. Over a median 10-year follow-up, 253 (18.2%) patients died, 39 (3.6%) suddenly. Analysis of the cardiac rhythm at the time of the 39 SD revealed sustained VTA in 9, asystole in 5, complete atrioventricular block in 1 and electromechanical dissociation in two patients. Non-cardiac causes were identified in the five patients with SD who underwent autopsies. Major conduction defects developed in 143 (19.3%) and sustained VTA in 26 (2.3%) patients. By Cox regression analysis, age, family history of SD and left bundle branch block were independent predictors of SD, while age, male sex, electrocardiographic conduction abnormalities, syncope, and atrial fibrillation were independent predictors of major conduction defects; non-sustained VTA was the only predictor of sustained VTA.
  
  
  CONCLUSIONS
  SD was a frequent mode of death in DM1, with multiple mechanisms involved. Major conduction defects were by far more frequent than sustained VTA, whose only independent predictor was a personal history of non-sustained VTA. ClinicalTrials.gov no: NCT01136330.
authors.affiliation: APHP, Cochin Hospital, Cardiology Department, Paris-Descartes, Sorbonne Paris Cité University, Paris, France.|APHP, Centre de Référence de pathologie neuromusculaire Paris-Est, Myology Institute, Neurology Department, Pitié-Salpêtrière Hospital, Paris, France.|Inserm, UMRS 974, Paris, France.
authors.forename: Karim
authors.initials: K
authors.lastname: Wahbi
authors.affiliation: Cardiology Department, Université François Rabelais, CHU Tours, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Babuty
authors.affiliation: INSERM, UMR1087, Université de Nantes, L'Institut du Thorax, CHU de Nantes, CIC, Centre de référence pour la prise en charge des maladies rythmiques héréditaires de Nantes, Nantes, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Probst
authors.affiliation: Cardiologie A, University Hospital, Lille, France.
authors.forename: Ludivine
authors.initials: L
authors.lastname: Wissocque
authors.affiliation: University Hospital of Caen, Caen, France.
authors.forename: Fabien
authors.initials: F
authors.lastname: Labombarda
authors.affiliation: INSERM U1153, 1 Place du Parvis Notre Dame, 75004 Paris, France; Université Paris Descartes - Sorbonne Paris Cité, Paris, France; Centre d'Epidémiologie Clinique, Hôpital Hôtel-Dieu, APHP, Paris, France.
authors.forename: Raphaël
authors.initials: R
authors.lastname: Porcher
authors.affiliation: APHP, Centre de Référence de pathologie neuromusculaire Paris-Est, Myology Institute, Neurology Department, Pitié-Salpêtrière Hospital, Paris, France.
authors.forename: Henri Marc
authors.initials: HM
authors.lastname: Bécane
authors.affiliation: InParys Clinical Research Group, Saint Cloud, France.
authors.forename: Arnaud
authors.initials: A
authors.lastname: Lazarus
authors.affiliation: APHP, Centre de Référence de pathologie neuromusculaire Paris-Est, Myology Institute, Neurology Department, Pitié-Salpêtrière Hospital, Paris, France.
authors.forename: Anthony
authors.initials: A
authors.lastname: Béhin
authors.affiliation: APHP, Centre de Référence de pathologie neuromusculaire Paris-Est, Myology Institute, Neurology Department, Pitié-Salpêtrière Hospital, Paris, France.|Pierre et Marie Curie-Paris 6 University, Paris, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Laforêt
authors.affiliation: APHP, Centre de Référence de pathologie neuromusculaire Paris-Est, Myology Institute, Neurology Department, Pitié-Salpêtrière Hospital, Paris, France.
authors.forename: Tanya
authors.initials: T
authors.lastname: Stojkovic
authors.affiliation: Cardiology Department, Université François Rabelais, CHU Tours, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Clementy
authors.affiliation: INSERM, UMR1087, Université de Nantes, L'Institut du Thorax, CHU de Nantes, CIC, Centre de référence pour la prise en charge des maladies rythmiques héréditaires de Nantes, Nantes, France.|Laboratoire d'Explorations Fonctionnelles, CHU de Nantes, Nantes, France.
authors.forename: Aurélie Pattier
authors.initials: AP
authors.lastname: Dussauge
authors.affiliation: INSERM, UMR1087, Université de Nantes, L'Institut du Thorax, CHU de Nantes, CIC, Centre de référence pour la prise en charge des maladies rythmiques héréditaires de Nantes, Nantes, France.
authors.forename: Jean Baptiste
authors.initials: JB
authors.lastname: Gourraud
authors.affiliation: Centre de Référence des Maladies Neuromusculaires Rares de l'Enfant et de l'Adulte Nantes-Angers, CHU de Nantes, Nantes, France.
authors.forename: Yann
authors.initials: Y
authors.lastname: Pereon
authors.affiliation: Clinique neurologique et centre de référence des maladies rares neuromusculaires, hôpital Roger-Salengro, CHRU de Lille, rue Emile-Laine, Lille, France.
authors.forename: Arnaud
authors.initials: A
authors.lastname: Lacour
authors.affiliation: Centre de compétences des pathologies neuromusculaires, CHU de Caen, Caen, France.
authors.forename: Françoise
authors.initials: F
authors.lastname: Chapon
authors.affiliation: University Hospital of Caen, Caen, France.
authors.forename: Paul
authors.initials: P
authors.lastname: Milliez
authors.affiliation: Cardiologie A, University Hospital, Lille, France.
authors.forename: Didier
authors.initials: D
authors.lastname: Klug
authors.affiliation: APHP, Centre de Référence de pathologie neuromusculaire Paris-Est, Myology Institute, Neurology Department, Pitié-Salpêtrière Hospital, Paris, France.|Pierre et Marie Curie-Paris 6 University, Paris, France.
authors.forename: Bruno
authors.initials: B
authors.lastname: Eymard
authors.affiliation: APHP, Cochin Hospital, Cardiology Department, Paris-Descartes, Sorbonne Paris Cité University, Paris, France.|APHP, Centre de Référence de pathologie neuromusculaire Paris-Est, Myology Institute, Neurology Department, Pitié-Salpêtrière Hospital, Paris, France.|Inserm, UMRS 974, Paris, France.
authors.forename: Denis
authors.initials: D
authors.lastname: Duboc
mesh_terms: D000328:Adult; D000367:Age Factors; D054537:Atrioventricular Block / Q000209:etiology / Q000401:mortality; D002037:Bundle-Branch Block / Q000209:etiology / Q000401:mortality; D000075224:Cardiac Conduction System Disease / Q000209:etiology* / Q000401:mortality; D002304:Cardiac Pacing, Artificial; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D017147:Defibrillators, Implantable; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000150:complications* / Q000401:mortality; D010375:Pedigree; D012189:Retrospective Studies; D017180:Tachycardia, Ventricular / Q000209:etiology / Q000401:mortality
keywords: Implantable cardioverter defibrillator; Myotonic dystrophy cardiomyopathy; Preventive pacing; Sudden cardiac death; Ventricular tachyarrhythmia; Ventricular tachycardia