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Cardiac involvement in myotonic dystrophy: a nationwide cohort study.
- Source :
-
European heart journal [Eur Heart J] 2014 Aug 21; Vol. 35 (32), pp. 2158-64. Date of Electronic Publication: 2014 Apr 16. - Publication Year :
- 2014
-
Abstract
- Aims: To quantify the association between myotonic dystrophy (DM) and cardiac disease in a nationwide cohort.<br />Methods and Results: We identified a nationwide cohort of 1146 DM patients (period 1977-2011) using the National Patient Registry (NPR) and a subcohort of 485 patients who had undergone genetic testing for DM1. Information on incident cardiac diseases was obtained from the NPR. We estimated standardized incidence ratios (SIRs) of cardiac disease compared with the background population, overall and according to selected diagnostic subgroups (cardiomyopathy, heart failure, conduction disorders, arrhythmias, and device implantation). In the DM cohort, SIR for any cardiac disease was 3.42 [95% confidence interval (CI) 3.01-3.86]; for a cardiac disease belonging to the selected subgroups 6.91 (95% CI: 5.93-8.01) and for other cardiac disease 2.59 (95% CI: 2.03-3.25). For a cardiac disease belonging to the selected subgroups, the risk was particularly high in the first year after DM diagnosis [SIR 15.4 (95% CI: 10.9-21.3)] but remained significantly elevated in subsequent years [SIR 6.07 (95% CI: 5.11-7.16]). The risk was higher in young cohort members [e.g. 20-39 years: SIR 18.1 (95% CI: 12.3-25.8)] compared with older [e.g. 60-79 years: SIR 3.99 (95% CI: 2.98-5.23)] but remained significantly increased in all age categories. Results were similar in separate analyses of the genetically confirmed DM1 patients.<br />Conclusion: Myotonic dystrophy is strongly associated with cardiac disease. The risk is pronounced in the young and remains elevated throughout life, stressing the importance of lifelong cardiac follow-up from time of DM diagnosis.<br /> (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.)
- Subjects :
- Adolescent
Adult
Aged
Aged, 80 and over
Child
Child, Preschool
Cohort Studies
Cross-Sectional Studies
Denmark epidemiology
Female
Heart Diseases epidemiology
Humans
Incidence
Infant
Infant, Newborn
Male
Middle Aged
Myotonic Dystrophy epidemiology
Risk Factors
Young Adult
Heart Diseases etiology
Myotonic Dystrophy complications
Subjects
Details
- Language :
- English
- ISSN :
- 1522-9645
- Volume :
- 35
- Issue :
- 32
- Database :
- MEDLINE
- Journal :
- European heart journal
- Publication Type :
- Academic Journal
- Accession number :
- 24742887
- Full Text :
- https://doi.org/10.1093/eurheartj/ehu157