Arrhythmias in Myotonic Muscular Dystrophy

Completed

• Conditions: Muscular Dystrophy; Arrhythmia; Sudden Cardiac Death
• Interventions: Other: Screening

• Registration Number: NCT00622453
• Lead Sponsor: Indiana University School of Medicine

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.

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 Timeline

• Study Start: 1996-09
• Primary Completion: 2006-01
• Study First Submitted: 2008-02-13
• Study First Submitted QC: 2008-02-13
• Study First Posted: 2008-02-25
• Study Completion: 2015-02
• Status Verified: 2018-02
• Last Update Submitted: 2018-02-13
• Last Update Posted: 2018-02-14

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 448

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.

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Registry of Arrhythmias	Screening	Screening of individuals with myotonic muscular dystrophy to evaluate the utility of non-invasive electrocardiographic screening methods and history in predicting serious arrhythmic events.

Primary Outcome Measures

Name	Time	Method
Evaluate incidence of arrhythmias in myotonic muscular dystrophy	3 years

Secondary Outcome Measures

Name	Time	Method
Evaluate with diagnostic non-invasive electrocardiogram (ECG)	3 Years

Trial Locations

Locations (1)

Krannert Institute of Cardiology; 🇺🇸 Indianapolis, Indiana, United States