Gossios, TD;
Providencia, R;
Creta, A;
Segal, OR;
Nikolenko, N;
Turner, C;
Lopes, LR;
... Savvatis, K; + view all
(2022)
An overview of heart rhythm disorders and management in myotonic dystrophy type 1.
Heart Rhythm
, 19
(3)
pp. 497-504.
10.1016/j.hrthm.2021.11.028.
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Abstract
Myotonic dystrophy type 1 (DM1) is the most common adult form of muscular dystrophy, presenting with a constellation of systemic findings secondary to a CTG triplet expansion of the noncoding region of the DMPK gene. Cardiac involvement is frequent, with conduction disease and supraventricular and ventricular arrhythmias being the most prevalent cardiac manifestations, often developing from a young age. The development of cardiac arrhythmias has been linked to increased morbidity and mortality, with sudden cardiac death well described. Strategies to mitigate risk of arrhythmic death have been developed. In this review, we outline the current knowledge on the pathophysiology of rhythm abnormalities in patients with myotonic dystrophy and summarize available knowledge on arrhythmic risk stratification. We also review management strategies from an electrophysiological perspective, attempting to underline the substantial unmet need to address residual arrhythmic risks for this population.
| Type: | Article |
|---|---|
| Title: | An overview of heart rhythm disorders and management in myotonic dystrophy type 1 |
| Location: | United States |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1016/j.hrthm.2021.11.028 |
| Publisher version: | http://dx.doi.org/10.1016/j.hrthm.2021.11.028 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | Science & Technology, Life Sciences & Biomedicine, Cardiac & Cardiovascular Systems, Cardiovascular System & Cardiology, Conduction disease, Electrophysiological study, Implantable cardioverter-defibrillator, Myotonic dystrophy, Pacemaker, Supraventricular arrhythmia, Ventricular arrhythmia, CARDIAC RESYNCHRONIZATION THERAPY, TERM-FOLLOW-UP, VENTRICULAR-TACHYCARDIA, PROTEIN-KINASE, MUSCULAR-DYSTROPHY, CONDUCTION SYSTEM, ATRIAL-FIBRILLATION, STEINERTS-DISEASE, BRUGADA SYNDROME, SUDDEN-DEATH |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Health Informatics |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10160236 |
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