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EditorialDOI Number : 10.36811/jcshd.2019.110005Article Views : 1581Article Downloads : 19

Consider cardiac and non-cardiac predictors of sudden death in myotonic dystrophy type 1

Josef Finsterer1* and Claudia Stöllberger2

1Krankenanstalt Rudolfstiftung, Messerli Institute, Veterinary University of Vienna, Austria, Europe
2Medical Department, Krankenanstalt Rudolfstiftung, Vienna, Austria, Europe

*Corresponding author:Finsterer Josef, Neurological Department, Krankenanstalt Rudolfstiftung, Univ. Prof. Dr. J. Finsterer, Schindlergasse 9/10, 1180 Vienna, Austria, Europe, Tel: +43-1-71165-72085; Fax: +43-1-4781711; Email: fifigs1@yahoo.de

Article Information

Aritcle Type: Editorial

Citation: Finsterer J, Claudia S. 2019 Consider cardiac and non-cardiac predictors of sudden death in myotonic dystrophy type 1. J Cardiovasc Surg Heart Dis. 1: 11-12.

Copyright: This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Copyright © 2019; Finsterer J

Publication history:

Received date: 12 January, 2019
Accepted date: 23 January, 2019
Published date: 24 January, 2019

In an article from the New England Journal of Medicine Groh et al. [1], found severe electrocardiographic abnormalities (sECGA) to predict sudden death (SD) among 406 patients with myotonic dystrophy 1 (MD1). However, it remains unclear if data were retrospectively or prospectively collected, why QT-prolongation, ventricular ectopic beats, or runs were not assessed as sECGA, how heart failure was defined, why only a quarter of the patients underwent imaging-studies, and why cardiac imaging-studies were assessed as events [2,3].

Was the “clinical diagnosis of tachyarrhythmia” only based on history or ECG-documentation? Which was the current medication of the included patients since it may affect their prognosis? How many patients experienced stroke or seizures or died from these diseases, which also cause SD? How to explain that the number of CTG repeats was associated with sECGA but not with mortality? Stating that mortality from respiratory failure may limit the benefit of cardioverter defibrillators is not based on the results since no patient with cardioverter defibrillator died from respiratory failure. To finally determine predictors of SD in MD1 more comprehensive cardiologic and non-cardiologic examinations are warranted. Loop-recording may be useful to detect malignant arrhythmias and to indicate implantation of a cardioverter-defibrillator [4].


  1. Groh WJ, Groh MR, Saha C, et al. 2008. Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1. N Engl J Med. 358: 2688-2697.[Ref.]
  2. Umeda Y, Ikeda U, Yamamoto J, et al. 1999. Myotonic dystrophy associated with QT prolongation and torsade de pointes. Clin Cardiol. 22: 136-138.[Ref.]
  3. Finsterer J, Stöllberger C. 2008. Primary myopathies and the heart. Scand Cardiovasc J. 42: 9-24.[Ref.]
  4. Finsterer J, Stöllberger C, Avanzini M, et al. 2008. Asymptomatic, nonsustained ventricular tachycardia in myotonic dystrophy type 1 detected with a loop recorder. Eur Neurol. 60: 109-111.[Ref.]

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