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Eur Heart J. 2017 Mar 7;38(10):751-758. doi: 10.1093/eurheartj/ehw569.

Incidence and predictors of sudden death, major conduction defects and sustained ventricular tachyarrhythmias in 1388 patients with myotonic dystrophy type 1.

Author information

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.
Cardiology Department, Université François Rabelais, CHU Tours, France.
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.
Cardiologie A, University Hospital, Lille, France.
University Hospital of Caen, Caen, France.
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.
InParys Clinical Research Group, Saint Cloud, France.
Pierre et Marie Curie-Paris 6 University, Paris, France.
Laboratoire d'Explorations Fonctionnelles, CHU de Nantes, Nantes, France.
Centre de Référence des Maladies Neuromusculaires Rares de l'Enfant et de l'Adulte Nantes-Angers, CHU de Nantes, Nantes, France.
Clinique neurologique et centre de référence des maladies rares neuromusculaires, hôpital Roger-Salengro, CHRU de Lille, rue Emile-Laine, Lille, France.
Centre de compétences des pathologies neuromusculaires, CHU de Caen, Caen, France.



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).

Methods and 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.


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. no: NCT01136330.


Implantable cardioverter defibrillator; Myotonic dystrophy cardiomyopathy; Preventive pacing; Sudden cardiac death; Ventricular tachyarrhythmia; Ventricular tachycardia

[Indexed for MEDLINE]

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