Risk of arrhythmia in type I myotonic dystrophy: the role of clinical and genetic variables

Abstract

Male sex, age and muscular disability were strongly associated with the development of arrhythmia in DM1. However, all of these variables were weak predictors of arrhythmic risk. These results suggest that other factors may be involved in the development of cardiac conduction abnormalities in DM1.

Cardiac manifestations of myotonic dystrophy type 1

“…In 2 (n = 419) of 6 studies a significant positive correlation was found between CTG repeat length and conduction abnormalities [20,24]. One study (n = 245) found a positive non-significant correlation between CTG repeat length and conduction abnormalities [9], whereas three smaller studies (in total n = 183) found no correlation between CTG repeat length and conduction abnormalities [12,17,18] (Table 3). Five (n = 770) of eight studies elaborated on the correlation between CTG repeat length and arrhythmic events (supraventricular and ventricular arrhythmias).…”

“…In 3 (n = 365) of 7 studies, there was a significant positive correlation between the severity of muscular involvement and conduction system impairment [9,13,18]. One study (n = 406) found a non-significant positive association between severity of muscular impairment and conduction abnormalities.…”

“…In 1 (n = 342) of 5 studies there was a significant positive correlation between CTG repeat length and arrhythmic events [24]. Another 1 (n = 245) of 5 studies found a non--significant positive correlation between CTG-repeat length and arrhythmic events [9]. Three smaller studies (in total n = 183) found no correlation between CTG repeat length and arrhythmic events [12,17,18].…”

“…In 2 (n= 328) of 6 studies there was a positive correlation between neuromuscular involvement and arrhythmic events [9,18]. One study (n= 406) found a non-significant positive association between severe muscular impairment and arrhythmia [3].…”

“…The prevalence of PM or ICD implantations was 4.1% and 1.1%, respectively (Table 3). Especially one study (n= 245) had a high prevalence of patients with arrhythmia treated with PM or ICD (11.0% and 5.3%, respectively) [9]. In this study, all patients were examined with Holter and ECG (Table 3).…”