Arrhythmias in Myotonic Muscular Dystrophy

NCT00622453 | Completed DMC

• 1 other identifier: 9609-31
• Study Type: observational
• Target: 448
• Locations: 1 country
• Sites: 1

Brief 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. In this study, we intend to evaluate the utility of non-invasive electrocardiographic screening methods and history in predicting serious arrhythmic events.

Conditions

Muscular Dystrophy; Arrhythmia; Sudden Cardiac Death

Outcome Measures

Primary Outcomes (1)

• Evaluate incidence of arrhythmias in myotonic muscular dystrophy

  3 years

Secondary Outcomes (1)

• Evaluate with diagnostic non-invasive electrocardiogram (ECG)

  3 Years

Study Arms (1)

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

Interventions

Screening OTHER

Electrocardiography Blood Test

Also known as: ECG, Holter Monitor, Blood Test

Registry of Arrhythmias

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• 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.

You may qualify if:

• Age 18 and over
• Willing to sign informed consent
• Have a previous diagnosis of myotonic muscular dystrophy

You may not qualify if:

• Under age 18.
• Unwilling to sign consent.
• Unwilling to commit to long-term follow-up.

Sponsors & Collaborators

• Indiana University School of Medicine: lead
• Muscular Dystrophy Association: collaborator

Study Sites (1)

Krannert Institute of Cardiology

Indianapolis, Indiana, 46202, United States

Location

Related Publications (6)

• 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.

  PMID: 18565861 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.

  PMID: 14760317 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.

  PMID: 14510658 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.

  PMID: 12030525 RESULT

• 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.

  PMID: 22035077 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.

  PMID: 21146669 DERIVED

MeSH Terms

Conditions

Muscular Dystrophies; Arrhythmias, Cardiac; Death, Sudden, Cardiac

Interventions

Mass Screening; Hematologic Tests

Condition Hierarchy (Ancestors)

Muscular Disorders, Atrophic; Muscular Diseases; Musculoskeletal Diseases; Neuromuscular Diseases; Nervous System Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Heart Diseases; Cardiovascular Diseases; Pathologic Processes; Pathological Conditions, Signs and Symptoms; Heart Arrest; Death, Sudden; Death

Intervention Hierarchy (Ancestors)

Diagnostic Techniques and Procedures; Diagnosis; Health Surveys; Surveys and Questionnaires; Data Collection; Epidemiologic Methods; Investigative Techniques; Diagnostic Services; Preventive Health Services; Health Services; Health Care Facilities Workforce and Services; Health Care Evaluation Mechanisms; Quality of Health Care; Health Care Quality, Access, and Evaluation; Public Health; Environment and Public Health; Public Health Practice; Clinical Laboratory Techniques

Study Officials

• William Groh, MD

  Indiana School of Medicine

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: OTHER
• Time Perspective: OTHER
• Sponsor Type: OTHER

Study Record Dates

• First Submitted: February 13, 2008
• First Posted: February 25, 2008
• Study Start: September 1, 1996
• Primary Completion: January 1, 2006
• Study Completion: February 1, 2015
• Last Updated: February 14, 2018

Record last verified: 2018-02

Locations

US (1)