Cardiovascular manifestations of myotonic dystrophy.

Publisher: Elsevier Science Publishing Country of Publication: United States NLM ID: 9108337 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-2615 (Electronic) Linking ISSN: 10501738 NLM ISO Abbreviation: Trends Cardiovasc Med Subsets: MEDLINE

Publication: New York Ny : Elsevier Science Publishing
Original Publication: New York : Elsevier Science Pub. Co.,

Cardiac Pacing, Artificial*/adverse effects ; Cardiac Pacing, Artificial*/mortality ; Electric Countershock*/adverse effects ; Electric Countershock*/instrumentation ; Electric Countershock*/mortality; Arrhythmias, Cardiac/*therapy ; Death, Sudden, Cardiac/*prevention & control ; Myotonic Dystrophy/*epidemiology; Animals ; Arrhythmias, Cardiac/genetics ; Arrhythmias, Cardiac/mortality ; Arrhythmias, Cardiac/physiopathology ; Death, Sudden, Cardiac/epidemiology ; Defibrillators, Implantable ; Genetic Predisposition to Disease ; Humans ; Myotonic Dystrophy/genetics ; Myotonic Dystrophy/mortality ; Myotonic Dystrophy/physiopathology ; Pacemaker, Artificial ; Prevalence ; Risk Factors ; Treatment Outcome

Patients with myotonic dystrophy, the most common neuromuscular dystrophy in adults, have a high prevalence of arrhythmic complications with increased cardiovascular mortality and high risk for sudden death. Sudden death prevention is central and relies on annual follow-up and prophylactic permanent pacing in patients with conduction defects on electrocardiogram and/or infrahisian blocks on electrophysiological study. Implantable cardiac defibrillator therapy may be indicated in patients with ventricular tachyarrhythmia.
(Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.)

Comment in: Trends Cardiovasc Med. 2020 May;30(4):239-240. (PMID: 31320264)

Date Created: 20190620 Date Completed: 20201021 Latest Revision: 20201021

20240829

10.1016/j.tcm.2019.06.001

31213350