Document Detail

Usefulness of clinical and electrocardiographic data for predicting adverse cardiac events in patients with myotonic dystrophy.
MedLine Citation:
PMID:  19214296     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Myotonic dystrophy type 1 (DM1) has been associated with an increased risk of sudden death, either by heart block or malignant ventricular arrhythmias. Identifying patients at risk remains difficult and no consensus has been reached regarding the best approach for follow-up and prevention of sudden death. OBJECTIVES: To identify noninvasive clinical and electrocardiographic predictors of adverse cardiac events in patients with DM1. METHODS: Clinical and serial electrocardiographic data on 428 patients with a DNA-proven diagnosis of DM1, followed during a mean period of 11.7 years, were reviewed. Variables associated with adverse cardiac events were identified. RESULTS: Eleven patients (2.6%) experienced sudden death and 13 (3.0%) required implantation of a pacemaker. On univariate analysis, adverse events were associated with advancing age, prolongation of the PR, QRS and corrected QT (QTc) intervals, as well as the degree of neuromuscular impairment. No such relationship was found with the extent of genetic anomaly (number of cytosine-thymine-guanine repeats). However, multivariate analysis using Cox proportional hazards models showed that only baseline PR and QTc intervals were significantly linked to the end points of sudden death or pacemaker implantation; the age-adjusted RR was 3.7 (95% CI 1.5 to 8.6) if baseline PR was 200 ms or longer (P=0.003), and 3.0 (95% CI 1.0 to 8.8) if the baseline QTc was 450 ms or longer (P=0.047). CONCLUSIONS: In a large unselected cohort of 428 patients with DM1, the cumulative incidence of sudden death was relatively low, and the delayed conduction on surface electrocardiogram was found to be potentially helpful for identifying patients at risk for sudden death or pacemaker implantation.
Robert Breton; Jean Mathieu
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Canadian journal of cardiology     Volume:  25     ISSN:  1916-7075     ISO Abbreviation:  Can J Cardiol     Publication Date:  2009 Feb 
Date Detail:
Created Date:  2009-02-13     Completed Date:  2009-03-27     Revised Date:  2010-09-23    
Medline Journal Info:
Nlm Unique ID:  8510280     Medline TA:  Can J Cardiol     Country:  Canada    
Other Details:
Languages:  eng     Pagination:  e23-7     Citation Subset:  IM    
Department of Cardiology, Clinical Research Unit, Chicoutimi Hospital, Saguenay, Quebec.
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MeSH Terms
Aged, 80 and over
Analysis of Variance
Arrhythmias, Cardiac / complications,  etiology*,  mortality,  physiopathology
Child, Preschool
Cohort Studies
Confidence Intervals
Death, Sudden, Cardiac / etiology*
Heart Block / complications,  etiology*,  mortality,  physiopathology
Heart Conduction System / physiopathology*
Middle Aged
Myotonic Dystrophy / complications*,  mortality,  physiopathology
Predictive Value of Tests
Proportional Hazards Models
Risk Assessment
Risk Factors
Young Adult

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