Cardiac involvement is recorded in about 80% of patients affected by myotonic dystrophy type 1 (DM1). The prevalence of cardiac conduction abnormalities is well described. Data regarding the prevalence of atrial fibrillation (AF) are still conflicting. The primary objective of this review was to assess the prevalence of AF in DM1. The secondary aim was to examine the association of clinical features with AF, 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, 2002 and January 30, 2020, assessing the prevalence of AF in DM1 population. Retrospective/prospective cohort studies and case series describing the prevalence of atrial fibrillation evaluated by periodic electrocardiogram (ECG) and/or ECG Holter 24 h, external loop recording (ELR) and implantable devices interrogation in DM1 patients were included. Case reports, simple reviews, commentaries and editorials were excluded. Thirteen reports fulfilled eligibility criteria and were included in our systematic review. According to the results from all the evaluated studies, the mean prevalence of AF in DM1 patients was 10.9% (n = 404) in 3677 DM1 patients. Male sex, conduction defects, echocardiographic findings of prolonged atrial electromechanical delay seem to be strongly associated with atrial fibrillation, representing factors favoring its onset. DM1 patients who develop AF seem to have a higher risk of cardiovascular and non-cardiovascular death. Further studies are needed to assess the prevalence of AF in DM1 patients and to investigate ECG abnormalities and other clinical features associated with this condition.
Keywords: Arrhythmias; Atrial fibrillation; Myotonic dystrophy type 1; Prevalence; Steinert disease.
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