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J Card Fail. 2019 Aug 12. pii: S1071-9164(19)30204-0. doi: 10.1016/j.cardfail.2019.07.548. [Epub ahead of print]

Prevalence of left ventricular systolic dysfunction in myotonic dystrophy type 1: a systematic review.

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Chair of Cardiology, Department of Translational Medical Sciences, University of Campania - Monaldi Hospital, Naples, Italy. Electronic address:
Chair of Cardiology, Department of Translational Medical Sciences, University of Campania - Monaldi Hospital, Naples, Italy.
Cardiomiology and Medical Genetics, Department of Experimental Medicine, University of Campania, Naples, Italy.
Cardiac Electrophysiology, The University of Pennsylvania Perelman School of Medicine, Philadelphia, USA.


Cardiac involvement is recorded in about 80% of patients affected by myotonic dystrophy type 1 (DM1). The prevalence of cardiac conduction abnormalities and arrhythmias is well described. Data regarding the prevalence of left ventricle systolic dysfunction (LVSD) and heart failure (HF) are still conflicting. The primary objective of this review was to assess the prevalence of LVSD and HF in DM1. The secondary aim was to examine the association of clinical features with LVSD, to detect predisposing and/or influencing prognosis factors. A systematic search was developed in MEDLINE, EMBASE, Cochrane Register of Controlled Trials, and Web of Science databases, to identify original reports between January 1, 2009 and September 30, 2017, assessing the prevalence of LVSD and HF in DM1 population. Retrospective and prospective cohort studies, and case series describing the prevalence of LVSD, evaluated by echocardiography, and HF in DM1 patients were included. Case reports, simple reviews, commentaries and editorials were excluded. Seven studies were identified as eligible, of which 1 was a retrospective population-based cohort study, and 6 were retrospective single center-based cohort studies. Echocardiographic data on LV function were available for 647 out of 876 DM1 patients included in the analysis. The prevalence of LVSD in DM1 patients, defined as LVEF < 55%, was 13.8%, 4.5-fold higher than general population. DM1 patients with LVSD were older, more likely male, had longer baseline atrioventricular and intraventricular conduction time duration, higher incidence of atrial arrhythmias, and were more likely to have undergone device implantation. Also symptomatic HF is more prevalent in DM1 patients despite their limited level of physical activity. Further studies are needed to evaluate the prevalence of LVSD and HF among DM1 patients and to investigate ECG abnormalities and other clinical features associated with this condition.


Heart failure; Left ventricle systolic dysfunction; Myotonic dystrophy type 1

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