title: Cardiologic predictors of sudden death in patients with myotonic dystrophy type 1.
abstract: The aim of this study was to analyze survival, causes of death and cardiologic predictors of sudden death in a large cohort of patients with myotonic dystrophy type 1 (DM1). The study was comprised of 171 adult DM1 patients hospitalized at the Neurology Clinic in a 20-year period. Severe electrocardiographic (ECG) abnormality included at least one of the following: rhythm other than sinus, PR interval of ≥240 ms, QRS complex duration of 120 ms or more, and second-degree or third-degree atrioventricular (AV) block. Survival data were analyzed by the Kaplan-Meier test, log-rank test and Cox regression analysis. During the mean follow-up period of 9.4±5.4 years, a pacemaker was implanted in 5.8% of DM1 patients and 14% of patients died. The mean age at death was 55.6±12.5 years. The most common causes of death in our cohort were sudden death (41.7%) and respiratory failure (29.2%). The presence of palpitations (hazard ratio [HR]=4.7, p<0.05) and increased systolic blood pressure (HR=9.8, p<0.05) were significant predictors of sudden death. Among ECG parameters, severe ECG abnormality (HR=4.7, p<0.05), right bundle branch block (RBBB; HR=3.9, p<0.05) and bifascicular block (HR=5.8, p<0.05) were significant predictors of sudden death.
authors.affiliation: Neurology Clinic, Clinical Centre of Serbia, School of Medicine, University of Belgrade, Dr Subotica 6, 11000 Belgrade, Serbia.
authors.forename: Vidosava
authors.initials: V
authors.lastname: Rakocevic Stojanovic
authors.forename: Stojan
authors.initials: S
authors.lastname: Peric
authors.forename: Teodora
authors.initials: T
authors.lastname: Paunic
authors.forename: Sanja
authors.initials: S
authors.lastname: Pavlovic
authors.forename: Edita
authors.initials: E
authors.lastname: Cvitan
authors.forename: Ivana
authors.initials: I
authors.lastname: Basta
authors.forename: Marina
authors.initials: M
authors.lastname: Peric
authors.forename: Milena
authors.initials: M
authors.lastname: Milicev
authors.forename: Dragana
authors.initials: D
authors.lastname: Lavrnic
mesh_terms: D000328:Adult; D017668:Age of Onset; D002423:Cause of Death; D016757:Death, Sudden, Cardiac / Q000453:epidemiology / Q000209:etiology*; D004562:Electrocardiography; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D009223:Myotonic Dystrophy / Q000150:complications* / Q000401:mortality*; D016016:Proportional Hazards Models; D012131:Respiratory Insufficiency / Q000453:epidemiology / Q000209:etiology; D012189:Retrospective Studies