This site became the new on June 19th. Learn more.
Show more Menu IMPORTANT: Listing of a study on this site does not reflect endorsement by the National Institutes of Health. Talk with a trusted healthcare professional before volunteering for a study. Read more... Menu IMPORTANT: Talk with a trusted healthcare professional before volunteering for a study. Read more... Menu
Give us feedback

Arrhythmias in Myotonic Muscular Dystrophy (DM1)

This study is ongoing, but not recruiting participants.
Muscular Dystrophy Association
Information provided by:
Indiana University Identifier:
First received: February 13, 2008
Last updated: July 29, 2015
Last verified: July 2015
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.

Condition Intervention
Muscular Dystrophy Arrhythmia Sudden Cardiac Death Other: Screening

Study Type: Observational
Official Title: A Registry of Arrhythmias in Myotonic Muscular Dystrophy

Resource links provided by NLM:

Further study details as provided by Indiana University:

Primary Outcome Measures:
  • Evaluate incidence of arrhythmias in myotonic muscular dystrophy [ Time Frame: 3 years ]

Secondary Outcome Measures:
  • Evaluate with diagnostic non-invasive electrocardiogram (ECG) [ Time Frame: 3 Years ]

Enrollment: 448
Study Start Date: September 1996
Estimated Study Completion Date: December 2017
Primary Completion Date: January 2006 (Final data collection date for primary outcome measure)
Groups/Cohorts Assigned Interventions
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.
Other: Screening
Electrocardiography Blood Test
Other Names:
  • ECG
  • Holter Monitor
  • Blood Test

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 This project is unique in that it characterizes a non-neurologic abnormality associated with a neuromuscular disease, myotonic muscular dystrophy.


Ages Eligible for Study:   18 Years and older   (Adult, Senior)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
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.

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.
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its identifier: NCT00622453

United States, Indiana
Krannert Institute of Cardiology
Indianapolis, Indiana, United States, 46202
Sponsors and Collaborators
Indiana University School of Medicine
Muscular Dystrophy Association
Principal Investigator: William Groh, MD Indiana School of Medicine
  More Information

Publications automatically indexed to this study by Identifier (NCT Number):
Responsible Party: William Groh, MD, Indiana University Identifier: NCT00622453     History of Changes
Other Study ID Numbers: 9609-31
Study First Received: February 13, 2008
Last Updated: July 29, 2015

Additional relevant MeSH terms:
Muscular Dystrophies
Myotonic Dystrophy
Myotonic Disorders
Death, Sudden, Cardiac
Muscular Disorders, Atrophic
Muscular Diseases
Musculoskeletal Diseases
Neuromuscular Diseases
Nervous System Diseases
Genetic Diseases, Inborn
Pathologic Processes
Heart Arrest
Heart Diseases
Cardiovascular Diseases
Death, Sudden
Heredodegenerative Disorders, Nervous System
Neurodegenerative Diseases processed this record on September 21, 2017