Document Detail

Cardiac manifestations of myotonic dystrophy type 1.
MedLine Citation:
PMID:  21917328     Owner:  NLM     Status:  MEDLINE    
AIMS: To estimate the degree of cardiac involvement regarding left ventricular ejection fraction, conduction abnormalities, arrhythmia, risk of sudden cardiac death (SCD) and the associations between cardiac involvement and cytosine-thymine-guanine (CTG)-repeat, neuromuscular involvement, age and gender in patients with myotonic dystrophy type 1 (MD1).
METHODS AND RESULTS: A Pub-Med search for the period 1980 to 2010 was performed according to specified criteria. Cardiac parameters including left ventricular ejection fraction (LVEF), conduction abnormalities and arrhythmia were compiled and only studies without ascertainment bias were included. Eighteen studies, 1828 MD1-patients, were included. The prevalence of atrioventricular block grade 1 (AVB1) was 28.2%, QTc>440 ms 22%, QRS>120 ms 19.9%, frequent ventricular premature contractions (VPC) 14.6%, atrial fibrillation/flutter (AF/AFL) 5%, right/left bundle branch block (RBBB/LBBB) 4.4/5.7% and non-sustained ventricular tachycardia (NSVT) 4.1%. Left ventricular systolic dysfunction (LVSD) was reported in 7.2% of the patients. There was an overall positive association between CTG-repeat size and cardiac involvement and between the degree of neuromuscular and cardiac involvement. Male gender and age were positively associated with arrhythmia and conduction abnormalities. The prevalence of pacemaker- (PM) and implantable cardioverter defibrillator-(ICD) implantations were 4.1% and 1.1%, respectively. The risk of SCD in this MD1-population was 0.56% per year.
CONCLUSION: MD1-patients have a high level of cardiac morbidity and mortality, strongly emphasizing the need of pre-symptomatic screening for arrhythmia and heart failure, as effective and well-documented preventive means are available.
Helle Petri; John Vissing; Nanna Witting; Henning Bundgaard; Lars Køber
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review     Date:  2011-09-13
Journal Detail:
Title:  International journal of cardiology     Volume:  160     ISSN:  1874-1754     ISO Abbreviation:  Int. J. Cardiol.     Publication Date:  2012 Oct 
Date Detail:
Created Date:  2012-09-11     Completed Date:  2013-01-28     Revised Date:  2013-11-14    
Medline Journal Info:
Nlm Unique ID:  8200291     Medline TA:  Int J Cardiol     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  82-8     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.
Department of Cardiology, National University Hospital Rigshospitalet, Copenhagen, Denmark.
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MeSH Terms
Heart Diseases / etiology*
Myotonic Dystrophy / complications*
Comment In:
Int J Cardiol. 2013 Sep 30;168(2):1541   [PMID:  23276392 ]
Int J Cardiol. 2013 Sep 1;167(5):2322-3   [PMID:  23181994 ]

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