Muscular DystrophyDM1NCT00622453
Arrhythmias in Myotonic Muscular Dystrophy
An observational study of Muscular Dystrophy and Arrhythmia, run by Indiana University at 1 site in United States.
Trial summary
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. Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Muscular Dystrophy, Arrhythmia, Sudden Cardiac Death
- What is being tested?
- Screening
- How many people
- 448 participants
- Where
- 1 siteUnited States
- Study dates
- Sep 1996to Feb 2015
- Phase
- Observational
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
Study population: 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.
Who may be able to join
- Age 18 and over
- Willing to sign informed consent
- Have a previous diagnosis of myotonic muscular dystrophy
Who cannot join
- Under age 18.
- Unwilling to sign consent.
- Unwilling to commit to long-term follow-up.
Trial locations and contacts
1 site in 1 country.
United States1 site
Krannert Institute of Cardiology
Indianapolis, Indiana, 46202
About this study
Official title: 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.
Read the detailed description
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.
- Conditions
- Muscular Dystrophy, Arrhythmia, Sudden Cardiac Death
- Being tested
- Screening
- Medical subject headings
- Muscular Dystrophies, Arrhythmias, Cardiac, Death, Sudden, Cardiac
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
ScreeningOther
Also called ECG, Holter Monitor, Blood Test
Electrocardiography Blood Test
| Study group | Role | What they receive |
|---|---|---|
| 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. Receives: Other: Screening |
What is being measured
The primary outcome is the main question the study is designed to answer.
Evaluate incidence of arrhythmias in myotonic muscular dystrophy
Measured: 3 years
Secondary outcomes (1)
Evaluate with diagnostic non-invasive electrocardiogram (ECG)
Measured: 3 Years
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted25 Feb 2008 (estimated)
- Study startSep 1996
- Primary completionJan 2006
- Study completionFeb 2015
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (6)Papers the sponsor has linked to this study.
- Other trials for Muscular DystrophyStudies of the same condition, open and under way.
- More trials run by Indiana UniversityThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Other
- Time perspective
- Other
- Enrolment
- 448 participants (actual)
Sponsor and investigators
- Lead sponsor
- Indiana University
- Sponsor type
- University, hospital or other
- Collaborators
- Muscular Dystrophy Association
- Investigators
- William Groh, MD, Principal investigator, Indiana School of Medicine
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT00622453
- Sponsor's protocol ID
- 9609-31
- Acronym
- DM1
- Status last verified
- Feb 2018
- First submitted
- 13 Feb 2008
- Last updated
- 14 Feb 2018
- Results posted
- No
- Data monitoring committee
- Yes
Publications
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.
PubMed 18565861Bhakta 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.
PubMed 14760317Hardin 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.
PubMed 14510658Groh 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.
PubMed 12030525Bhakta 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.
PubMed 22035077Bhakta 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.
PubMed 21146669
Study at a glance
From the registry record, last updated by the sponsor on 14 Feb 2018. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- A Registry of Arrhythmias in Myotonic Muscular Dystrophy
- ClinicalTrials.gov ID
- NCT00622453
- Acronym / sponsor ID
- DM1 / 9609-31
- Conditions
- Muscular Dystrophy, Arrhythmia, Sudden Cardiac Death
- Study type
- Observational
- Recruitment status
- Completed
- Enrolment
- 448 participants (actual)
- Lead sponsor
- Indiana UniversityUniversity, hospital or other
- Collaborators
- Muscular Dystrophy Association
- Study start
- Sep 1996
- Primary completion
- Jan 2006 (actual)
- Study completion
- Feb 2015 (actual)
- First posted
- 25 Feb 2008 (estimated)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
Detailed description
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.
Study design
- Observational model
- Other
- Time perspective
- Other
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Registry of Arrhythmias | – | Other: ScreeningScreening of individuals with myotonic muscular dystrophy to evaluate the utility of non-invasive electrocardiographic screening methods and history in predicting serious arrhythmic events. |
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- Study population
- 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.
Key inclusion criteria (3 listed)
- Age 18 and over
- Willing to sign informed consent
- Have a previous diagnosis of myotonic muscular dystrophy
Key exclusion criteria (3 listed)
- Under age 18.
- Unwilling to sign consent.
- Unwilling to commit to long-term follow-up.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| ScreeningAlso: ECG, Holter Monitor, Blood TestElectrocardiography Blood Test | Other | Registry of Arrhythmias |
Primary outcome
Evaluate incidence of arrhythmias in myotonic muscular dystrophy
Time frame: 3 years
1 secondary outcome
Evaluate with diagnostic non-invasive electrocardiogram (ECG)
Time frame: 3 Years
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 14 Feb 2018.
- Results publication: PubMed 18565861Groh 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.
- Results publication: PubMed 14760317Bhakta 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.
- Results publication: PubMed 14510658Hardin 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.
- Results publication: PubMed 12030525Groh 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.
- Derived from this study: PubMed 22035077Bhakta 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.
- Derived from this study: PubMed 21146669Bhakta 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.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
William Groh, MD principal investigator
Indiana School of Medicine
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Muscular Dystrophy and Arrhythmia, run by Indiana University at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 3 inclusion and 3 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 14 Feb 2018. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Evaluate incidence of arrhythmias in myotonic muscular dystrophy (time frame: 3 years). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT00622453, which also lists 6 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 14 Feb 2018.