Arrhythmias in Myotonic Muscular Dystrophy
| Status: | Completed |
|---|---|
| Conditions: | Cardiology, Neurology |
| Therapuetic Areas: | Cardiology / Vascular Diseases, Neurology |
| Healthy: | No |
| Age Range: | 18 - Any |
| Updated: | 2/16/2018 |
| Start Date: | September 1996 |
| End Date: | February 2015 |
A Registry of Arrhythmias in Myotonic Muscular Dystrophy
Adult myotonic muscular dystrophy (Steinert's disease) is the most common inherited
neuromuscular disorder. Cardiac rhythm disturbances occur frequently in this disease state
and may be responsible for up to one-third of deaths. In this study, we intend to evaluate
the utility of non-invasive electrocardiographic screening methods and history in predicting
serious arrhythmic events.
neuromuscular disorder. Cardiac rhythm disturbances occur frequently in this disease state
and may be responsible for up to one-third of deaths. In this study, we intend to evaluate
the utility of non-invasive electrocardiographic screening methods and history in predicting
serious arrhythmic events.
The long term objectives of this population study is a more defined natural history, optimal
diagnostic testing methodology, and methods of therapy for arrhythmias in individuals with
myotonic muscular dystrophy. The goal is a more adequate definition of appropriate diagnosis
and therapy for arrhythmias in order to decrease the likelihood of cardiac morbidity and
mortality in this disorder.
The specific aims of the study involve an initial survey of individuals with myotonic
muscular dystrophy detailing multiple factors. Non-invasive electrocardiographic testing will
be done. Using this initial data and subsequent follow-up data collected yearly the cohort of
patients will be followed as to arrhythmia development over a minimum of five years and
likely longer with a long-term registry and evaluation of National Death Records and
Ancestry.com. This project is unique in that it characterizes a non-neurologic abnormality
associated with a neuromuscular disease, myotonic muscular dystrophy.
diagnostic testing methodology, and methods of therapy for arrhythmias in individuals with
myotonic muscular dystrophy. The goal is a more adequate definition of appropriate diagnosis
and therapy for arrhythmias in order to decrease the likelihood of cardiac morbidity and
mortality in this disorder.
The specific aims of the study involve an initial survey of individuals with myotonic
muscular dystrophy detailing multiple factors. Non-invasive electrocardiographic testing will
be done. Using this initial data and subsequent follow-up data collected yearly the cohort of
patients will be followed as to arrhythmia development over a minimum of five years and
likely longer with a long-term registry and evaluation of National Death Records and
Ancestry.com. This project is unique in that it characterizes a non-neurologic abnormality
associated with a neuromuscular disease, myotonic muscular dystrophy.
Inclusion Criteria:
1. Age 18 and over
2. Willing to sign informed consent
3. Have a previous diagnosis of myotonic muscular dystrophy
Exclusion Criteria:
1. Under age 18.
2. Unwilling to sign consent.
3. Unwilling to commit to long-term follow-up.
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