Abstract
Background: Myotonic dystrophy type 1 (MDl) is a multisystem disease with cardiac involvement. Ventricular tachycardia (VTJ is common in these patients (pts) and may be causally related to sudden death. The aim of OUT study was to evaluate in MD1 pts non invasive and invasive parameters that have been associated with clinical VT in other populations. Methods: Among 202 MD1 pts routinely followed in OUT Center, 44 pts (14 female, mean age: 50.4&10.8 years) presented with symptoms and/or bradytachyarrhythmias, underwent an invasive electmphysiologic (EP) evaluation. The EP study included programmed ventricular stimulation for VT induction. Time and frequency-domain heart rate variability (HRV) analysis was performed on 24.hour Halter electrocardiographic recordings in all MD1 pts and in 15 healthy age-matched controls. In addition, the presence of late ventricular potentials (LPs) was assessed by signal averaged ECG. Results: A significant decrease in SDNNi in MD1 Pts (70.7&29.1) compared to controls (122.2&46.6) was found. Furthermore, in MD1 pts, a statistically significant depression of the indexes of parasympathetic activity was detected by frequency domain HRV analysis, in comparison with the control group (HF 519.9&268.4 vs 997.3&289.4 msec’, ~~0.05; LF/HF ratio 2.6&0.6 vs 1.5&0.5, p<O.O5). LPs were present in the majority of MD1 pts (23144, 52%). Programmed ventricular stimulation reproducibly induced sustained VT in 11144 (25%) MD1 pts. Comparing inducible and non-inducible pts, no significant difference was observed in HRV and LPs parameters. Conclusions: Among MD1 pts with an indication for EP study, a high incidence of HRV abnormalities, LPs and VT inducibility was found. However, parameters of autonomic dysfunction and LPs were not predictive of inducibility of sustained VTs, suggesting that invasive and non invasive tests provide complementary information on the arrhythmia profile of MD1 pts. Further follow-up studies are needed to correlate these findings to the developement of clinical VTs.
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