Clinical Trial: RAMYD Study - Evaluation of Arrhythmic Risk in Myotonic Dystrophy

Study Status: Completed
Recruit Status: Unknown status
Study Type: Interventional

Official Title: Evaluation of Arrhythmic Risk in Myotonic Dystrophy Type I (DM 1)

Brief Summary: This is a prospective multicentric Italian study to evaluate the arrhythmic risk in myotonic dystrophy type 1.

Detailed Summary:

Myotonic dystrophy type 1 (DM1, Steinert disease) is a multisystem disorder that affects, beside muscle, several other organs, including the heart.

Cardiac involvement represents a major problem in the clinical management of patients, so that cardiac complications represent one of the primary causes of premature death in DM1. In particular there is a high incidence of sudden death, ranging from 2 to 30% of cases, so far principally related to the development of conduction blocks. However, literature reports of sudden death in patients implanted with pacemakers, as well as of spontaneous ventricular tachycardia would suggest a potential etiologic role also for ventricular arrhythmias. The lack of clinical research studies conducted on a large number of patients does not make available definite data regarding the etiology and the epidemiology of arrhythmic events in DM1. For the same reasons, other considerable topics, such as prognostic stratification of the arrhythmic risk and clinical management of life-threatening arrhythmias in DM1 patients, are still undefined.

To clarify these issues, the investigators propose a clinical research study performed on a large cohort of DM1 patients enrolled through a multicenter collaboration that also involves 5 cardiological-neurological Italian centres.

Aims of this study are:

  • To estimate the incidence of arrhythmias and to characterize the brady-tachyarrhythmic mechanisms underlying the occurrence of cardiac sudden death in DM1;
  • To verify by statistical analysis the reliability of data obtained from both non invasive and invasive diagnostic procedures as indexes useful for estimating the arrhythmic risk in DM1;
    • Sponsor: Catholic University of the Sacred Heart

    Current Primary Outcome:

    • Evaluate incidence of: major cardiac events (sudden death
    • resuscitated cardiac arrest
    • ventricular fibrillation
    • sustained ventricular tachycardia
    • sinoatrial and atrioventricular [AV] blocks)


    Original Primary Outcome:

    • Evaluate incidence of:
    • 1) major cardiac events (sudden death, resuscitated cardiac arrest, ventricular fibrillation, sustained ventricular tachycardia, sinoatrial and AV blocks).


    Current Secondary Outcome:

    • Evaluate with diagnostic non-invasive (standard electrocardiogram [ECG]
    • 24-hour monitoring ECG
    • signal-averaged ECG
    • echocardiography) and invasive procedures (electrophysiology study [EPS] and implantable loop recorders) the risk to develop cardiac arrhythmias in DM patients


    Original Secondary Outcome: Evaluate with diagnostic non-invasive (standard ECG, 24-hour monitoring ECG, signal-averaged ECG, echocardiography) and invasive procedures (EPS and implantable loop recorders) the risk to develop cardiac arrhythmias in DM patients.

    Information By: Catholic University of the Sacred Heart

    Dates:
    Date Received: August 4, 2005
    Date Started: January 2003
    Date Completion:
    Last Updated: August 24, 2005
    Last Verified: May 2005