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| Name | Class |
|---|---|
| Muscular Dystrophy Association | OTHER |
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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Registry of Arrhythmias | Screening of individuals with myotonic muscular dystrophy to evaluate the utility of non-invasive electrocardiographic screening methods and history in predicting serious arrhythmic events. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Screening | Other | Electrocardiography Blood Test |
|
| Measure | Description | Time Frame |
|---|---|---|
| Evaluate incidence of arrhythmias in myotonic muscular dystrophy | 3 years |
| Measure | Description | Time Frame |
|---|---|---|
| Evaluate with diagnostic non-invasive electrocardiogram (ECG) | 3 Years |
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Inclusion Criteria:
Exclusion Criteria:
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Patient Recruitment: Will take place at the 230 hospital-affiliated MDA neuromuscular clinics. Individuals with myotonic dystrophy will be identified in the MDA clinics these will be asked to participate in the study.
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| Name | Affiliation | Role |
|---|---|---|
| William Groh, MD | Indiana School of Medicine | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Krannert Institute of Cardiology | Indianapolis | Indiana | 46202 | United States |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 18565861 | Result | Groh WJ, Groh MR, Saha C, Kincaid JC, Simmons Z, Ciafaloni E, Pourmand R, Otten RF, Bhakta D, Nair GV, Marashdeh MM, Zipes DP, Pascuzzi RM. Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1. N Engl J Med. 2008 Jun 19;358(25):2688-97. doi: 10.1056/NEJMoa062800. | |
| 14760317 | Result | Bhakta D, Lowe MR, Groh WJ. Prevalence of structural cardiac abnormalities in patients with myotonic dystrophy type I. Am Heart J. 2004 Feb;147(2):224-7. doi: 10.1016/j.ahj.2003.08.008. |
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| ID | Term |
|---|---|
| D009136 | Muscular Dystrophies |
| D001145 | Arrhythmias, Cardiac |
| D016757 | Death, Sudden, Cardiac |
| ID | Term |
|---|---|
| D020966 | Muscular Disorders, Atrophic |
| D009135 | Muscular Diseases |
| D009140 | Musculoskeletal Diseases |
| D009468 | Neuromuscular Diseases |
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| ID | Term |
|---|---|
| D008403 | Mass Screening |
| D006403 | Hematologic Tests |
| ID | Term |
|---|---|
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
| D006306 | Health Surveys |
| D011795 | Surveys and Questionnaires |
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| 14510658 | Result | Hardin BA, Lowe MR, Bhakta D, Groh WJ. Heart rate variability declines with increasing age and CTG repeat length in patients with myotonic dystrophy type 1. Ann Noninvasive Electrocardiol. 2003 Jul;8(3):227-32. doi: 10.1046/j.1542-474x.2003.08310.x. |
| 12030525 | Result | Groh WJ, Lowe MR, Zipes DP. Severity of cardiac conduction involvement and arrhythmias in myotonic dystrophy type 1 correlates with age and CTG repeat length. J Cardiovasc Electrophysiol. 2002 May;13(5):444-8. doi: 10.1046/j.1540-8167.2002.00444.x. |
| 22035077 | Derived | Bhakta D, Shen C, Kron J, Epstein AE, Pascuzzi RM, Groh WJ. Pacemaker and implantable cardioverter-defibrillator use in a US myotonic dystrophy type 1 population. J Cardiovasc Electrophysiol. 2011 Dec;22(12):1369-75. doi: 10.1111/j.1540-8167.2011.02200.x. Epub 2011 Oct 28. |
| 21146669 | Derived | Bhakta D, Groh MR, Shen C, Pascuzzi RM, Groh WJ. Increased mortality with left ventricular systolic dysfunction and heart failure in adults with myotonic dystrophy type 1. Am Heart J. 2010 Dec;160(6):1137-41, 1141.e1. doi: 10.1016/j.ahj.2010.07.032. |
| D009422 | Nervous System Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D006331 | Heart Diseases |
| D002318 | Cardiovascular Diseases |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D006323 | Heart Arrest |
| D003645 | Death, Sudden |
| D003643 | Death |
| D003625 | Data Collection |
| D004812 | Epidemiologic Methods |
| D008919 | Investigative Techniques |
| D003954 | Diagnostic Services |
| D011314 | Preventive Health Services |
| D006296 | Health Services |
| D005159 | Health Care Facilities Workforce and Services |
| D017531 | Health Care Evaluation Mechanisms |
| D011787 | Quality of Health Care |
| D017530 | Health Care Quality, Access, and Evaluation |
| D011634 | Public Health |
| D004778 | Environment and Public Health |
| D015980 | Public Health Practice |
| D019411 | Clinical Laboratory Techniques |