Document Detail

Age, conduction defects and restrictive lung disease independently predict cardiac events and death in myotonic dystrophy.
MedLine Citation:
PMID:  21640397     Owner:  NLM     Status:  Publisher    
OBJECTIVE: The aim of the study was to identify, in addition to conduction defects, possible predictors of cardiac events and death in patients with myotonic dystrophy (DM1). METHODS AND DESIGN: A retrospective observational cohort study was undertaken. Baseline clinical and non-invasive cardiac and respiratory investigations were obtained from 107 DM1 patients, who were regularly re-examined. Primary end-points were occurrence of cardiac events (pacemaker implantation or tachyarrhythmia) or death. Probability of an event was calculated using the Kaplan-Meier method, while contributing factors were assessed using univariate and multivariate (Cox model) analyses. RESULTS: Cardiac events occurred in 34 patients (29%). Age, muscular impairment, infantile onset, restrictive lung disease (RLD), ECG conduction defects, left ventricular ejection fraction (LVEF) below 50%, and arrhythmia detected during Holter monitoring were predictors of cardiac events. Multivariate analysis indicated that age, RLD, ECG conduction defects, Holter arrhythmia and LVEF remained independent predictors. Probability of cardiac events was 2.5% (5%CI: 0-7%) at 1year and 6% (5%CI: 0-14%) at 3years in patients younger than 42years with normal ECG, Holter, LVEF and lung volumes. Advancing age, distal or proximal weakness and RLD characterized all non-survivors (n=14). CONCLUSION: Cardiac events or death are predicted not only by conduction defects or cardiomyopathy in DM1, but also by RLD, muscular disability and advancing age. Addition of these criteria should modulate time intervals for patient follow-up examinations. In young patients with normal baseline investigations, screening investigations every 2 or 3years seem to be sufficient.
Pierre Kaminsky; Béatrice Brembilla-Perrot; Lelia Pruna; Mathias Poussel; Bruno Chenuel
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-6-1
Journal Detail:
Title:  International journal of cardiology     Volume:  -     ISSN:  1874-1754     ISO Abbreviation:  -     Publication Date:  2011 Jun 
Date Detail:
Created Date:  2011-6-6     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8200291     Medline TA:  Int J Cardiol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.
Service de Médecine Interne et Maladies Orphelines, Centre Hospitalier Universitaire de Nancy, Hôpitaux de Brabois, and Faculté de Médecine, avenue de la Forêt de Haye, 54500 Vandoeuvre-Cedex, France; Centre de Référence des Maladies Neuromusculaires, Centre Hospitalier Universitaire de Nancy, Hôpitaux de Brabois, and Faculté de Médecine, avenue de la Forêt de Haye, 54500 Vandoeuvre-Cedex, France.
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