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Europace Advance Access originally published online on October 29, 2007
Europace 2007 9(12):1158-1160; doi:10.1093/europace/eum236
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org

ICDS

‘Unexpected’ sudden death avoided by implantable cardioverter–defibrillator in Emery–Dreifuss patient

Pier Giorgio Golzio1,*, Amedeo Chiribiri1 and Fiorenzo Gaita2

1 Division of Cardiology, Department of Internal Medicine, University of Turin, Turin, Italy; 2 Department of Cardiology, Cardinal G. Massaia Hospital, Asti, Italy

A female patient just over 20 years of age developed first grade atrioventricular block, and later atrial fibrillation. When she was 41 years old she was diagnosed with Emery-Dreifuss muscular dystrophy (EDMD). A VVIR pacemaker was implanted in 2002, replaced in 2003 with an ICD. Nine months later, during febrile illness, the patient experienced three appropriate ICD discharges. No further ICD interventions occurred. The transient course of arrhythmic activity and the possible influence of triggering factors lessen the role of electrophysiologic study to identify risk of sudden death, and suggest that in patients with EDMD requiring pacemaker implantation, an ICD would be more properly indicated.

Key Words: Sudden death, familial cardiomyopathy, arrhythmia, LMNA gene, ICD, primary prevention

* Corresponding author: Divisione Universitaria di Cardiologia, Ospedale San Giovanni Battista, Corso A.M. Dogliotti, 14, 10126 Torino, Italy. Tel: +39 0116636165; fax: +39 0116967053. E-mail address: pgolzio{at}molinette.piemonte.it pg.golzio{at}gmail.com pg.golzio{at}libero.it

Manuscript submitted 15 August 2007. Accepted after revision 26 September 2007.


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