Marie Lund1, Lars Jorge Diaz2, Mattis Flyvholm Ranthe2, Helle Petri3, Morten Duno4, Inger Juncker5, Hans Eiberg6, John Vissing7, Henning Bundgaard3, Jan Wohlfahrt2, Mads Melbye8. 1. Department of Epidemiology Research, Statens Serum Institut, Artillerivej 5, Copenhagen S, Denmark mxd@ssi.dk. 2. Department of Epidemiology Research, Statens Serum Institut, Artillerivej 5, Copenhagen S, Denmark. 3. Unit for Inherited Cardiac Diseases, Department of Cardiology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. 4. Department of Clinical Genetics, Molecular Genetic Laboratory, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. 5. Department of Clinical Genetics, Aarhus University Hospital, Aarhus, Denmark. 6. Department of Cellular and Molecular Medicine, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark. 7. Neuromuscular Research Unit, Department of Neurology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. 8. Department of Epidemiology Research, Statens Serum Institut, Artillerivej 5, Copenhagen S, Denmark Department of Medicine, Stanford School of Medicine, Stanford, CA, USA.
Abstract
AIMS: To quantify the association between myotonic dystrophy (DM) and cardiac disease in a nationwide cohort. 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. 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. Published on behalf of the European Society of Cardiology. All rights reserved.
AIMS: To quantify the association between myotonic dystrophy (DM) and cardiac disease in a nationwide cohort. METHODS AND RESULTS: We identified a nationwide cohort of 1146 DMpatients (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 DM1patients. 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. Published on behalf of the European Society of Cardiology. All rights reserved.
Authors: A F Best; J E Hilbert; L Wood; W B Martens; N Nikolenko; C Marini-Bettolo; H Lochmüller; P S Rosenberg; R T Moxley; M H Greene; S M Gadalla Journal: Eur J Neurol Date: 2018-09-16 Impact factor: 6.089
Authors: Alexandre Gamet; Bruno Degand; François Le Gal; Nicolas Bidegain; Anne Delaubier; Brigitte Gilbert-Dussardier; Luc Christiaens; Rodrigue Garcia Journal: Ann Noninvasive Electrocardiol Date: 2018-08-12 Impact factor: 1.468