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Europace. 2014 Dec;16(12):1814-20. doi: 10.1093/europace/euu141. Epub 2014 Jun 17.

Atrial arrhythmias in the young: early onset atrial arrhythmias preceding a diagnosis of a primary muscular dystrophy.

Author information

  • 1Arrhythmia Service, Division of Cardiology, University of Ottawa Heart Institute, Ottawa, ON, Canada K1Y 4W7.
  • 2Arrhythmia Service, Division of Cardiology, Loyola Center for Heart and Vascular Medicine, Park Ridge, 60153 IL, USA.
  • 3Arrhythmia Service, Division of Cardiology, Cleveland Clinic Foundation, 44106 Cleveland, OH, USA.
  • 4Arrhythmia Service, Division of Cardiology, University of Ottawa Heart Institute, Ottawa, ON, Canada K1Y 4W7 mgollob@ottawaheart.ca.



The aetiology of atrial arrhythmias in the otherwise healthy and young is usually unrecognized. We hypothesized that rare cases of atrial arrhythmias in the young may represent the initial manifestation of a muscular dystrophy syndrome.


We describe the clinical characteristics, disease progression, results of electrophysiological study, and genetic findings in four patients (age <40 years) presenting with idiopathic atrial arrhythmias who subsequently received a diagnosis of a muscular dystrophy syndrome. The mean age at presentation with atrial arrhythmias was 29.5 years (range, 21-37 years), and the mean delay to diagnosis of muscular dystrophy was 3.6 years (range, 0.5-6 years). Two patients received a subsequent diagnosis of myotonic dystrophy type 1 and 2 a diagnosis of Emery-Dreifuss muscular dystrophy. Disease-causing genetic defects were identified in all four patients. One patient underwent catheter ablation of atrial flutter, experiencing improvement in arrhythmia symptoms. Two patients required device therapy, each receiving cardiac resynchronization therapy-defibrillator implantation for progressive left ventricular dysfunction.


Early onset atrial arrhythmias may be the first clinical manifestation of a muscular dystrophy syndrome. Appropriate clinical assessment and surveillance may uncover this primary cause and provide an opportunity for timely genetic counselling and family screening.

Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.


Atrial fibrillation; Genetics; Muscular dystrophy

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