Abstract
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.
Keywords
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Article info
Publication history
Published online: September 14, 2011
Accepted:
August 17,
2011
Received:
July 18,
2011
Footnotes
☆Funding: this work was supported by the Research Foundation of Rigshospitalet.
Identification
Copyright
© 2011 Elsevier Ireland Ltd. Published by Elsevier Inc. All rights reserved.
