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Karim Wahbi, Dominique Babuty, Vincent Probst, Ludivine Wissocque, Fabien Labombarda, Raphaël Porcher, Henri Marc Bécane, Arnaud Lazarus, Anthony Béhin, Pascal Laforêt, Tanya Stojkovic, Nicolas Clementy, Aurélie Pattier Dussauge, Jean Baptiste Gourraud, Yann Pereon, Arnaud Lacour, Françoise Chapon, Paul Milliez, Didier Klug, Bruno Eymard, Denis Duboc, Incidence and predictors of sudden death, major conduction defects and sustained ventricular tachyarrhythmias in 1388 patients with myotonic dystrophy type 1, European Heart Journal, Volume 38, Issue 10, 7 March 2017, Pages 751–758, https://doi.org/10.1093/eurheartj/ehw569
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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).
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. ClinicalTrials.gov no: NCT01136330.
