Myotonic Dystrophy Clinical Trial
Official title:
Sleep Breathing Disorders, a Main Trigger for Cardiac ARythmias in Type I Myotonic Dystrophy ?
Up to one-third of patients with myotonic dystrophy type 1 die suddenly mainly from arrhythmias. Sleep apnea is prevalent in myotonic dystrophy (DM1) patients. Among the serious complications from sleep apnea, the most alarming are arrhythmias and sudden cardiac death (SCD). Diagnosis of sleep apnea using simple tools in ambulatory cardiology practice may improve therapy of cardiac arrhythmias in patients with DM1
Obstructive sleep apnea (OSA) and central sleep apnea (CSA), the most common form of sleep
disordered breathing (SDB), are prevalent in patients with myotonic dystrophy type 1 (DM1).
Among the serious complications from sleep apnea, the most alarming are cardiovascular,
including arrhythmias and sudden cardiac death (SCD). Diagnosis of SDB using simple tools in
ambulatory cardiology practice may lead to an important primary or additional therapy to
supplement the use of drugs or devices in the treatment of cardiac arrhythmias.
We hypothesize that DM1 patients with severe oxygen desaturations (Oxygen desaturation index
>15/hour of sleep and/or cumulative time spent below 90% of SaO2 above 5% of time of
recording) will exhibit three fold more nocturnal arrhythmias compared to DM1 group without
oxygen desaturations during sleep.
During this project we will address the following aims:
- Is there a relationship between the severity of oxygen desaturations during sleep and
nocturnal arrhythmias? We will address this question in a prospective study with seven
nights of at home recordings with a multimodal holter EKG assessing together
arrhythmias, thoracic impedance (in order to estimate respiratory movements) and SaO2.
- The specific proarrhythmic role of REM sleep will be assessed during a single night full
polysomnography and multiple sleep latency tests (MSLT).
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