RAMYD Study - Evaluation of Arrhythmic Risk in Myotonic Dystrophy

The recruitment status of this study is unknown because the information has not been verified recently.
Verified May 2005 by Catholic University of the Sacred Heart.
Recruitment status was  Recruiting
Sponsor:
Collaborator:
Fondazione Telethon
Information provided by:
Catholic University of the Sacred Heart
ClinicalTrials.gov Identifier:
NCT00127582
First received: August 4, 2005
Last updated: August 24, 2005
Last verified: May 2005

August 4, 2005
August 24, 2005
January 2003
Not Provided
  • Evaluate incidence of: major cardiac events (sudden death
  • resuscitated cardiac arrest
  • ventricular fibrillation
  • sustained ventricular tachycardia
  • sinoatrial and atrioventricular [AV] blocks)
  • Evaluate incidence of:
  • 1) major cardiac events (sudden death, resuscitated cardiac arrest, ventricular fibrillation, sustained ventricular tachycardia, sinoatrial and AV blocks).
Complete list of historical versions of study NCT00127582 on ClinicalTrials.gov Archive Site
  • 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
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.
Not Provided
Not Provided
 
RAMYD Study - Evaluation of Arrhythmic Risk in Myotonic Dystrophy
Evaluation of Arrhythmic Risk in Myotonic Dystrophy Type I (DM 1)

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

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;
  • To identify more adequate therapeutic guidelines in order to prevent the occurrence of life-threatening arrhythmias.

The protocol of study includes:

  1. Clinical-genetic evaluation;
  2. Non invasive and invasive diagnostic cardiac procedures;
  3. The use of devices for diagnostic and therapeutic follow-up.
Interventional
Phase 3
Allocation: Non-Randomized
Endpoint Classification: Safety Study
Intervention Model: Single Group Assignment
Masking: Open Label
Primary Purpose: Treatment
  • Myotonic Dystrophy
  • Sudden Cardiac Death
  • Procedure: Electrophysiological study
  • Device: pacemaker (PM) implant, internal cardiac defibrillator (ICD) implant, loop-recorder implant
Not Provided

*   Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
 
Recruiting
537
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Inclusion Criteria:

  • Patient affected by myotonic dystrophy type I (MD1).
  • Patient willing to provide a signed informed consent.

Exclusion Criteria:

  • Age < 18 years old or >70 years old.
  • Ischemic cardiomyopathy
  • Cardiomyopathy due to chronic excess of alcohol consumption (>100 g\day)
  • Congenital heart disease
  • Acquired valvular heart disease
  • Metabolic cardiomyopathy: thyrotoxicosis, hypothyroidism, adrenal cortical insufficiency, pheochromocytoma, acromegaly
  • Familiar storage and infiltrative diseases (hemochromatosis, glycogen storage, Hurler’s syndrome, Niemann-Pick disease; primary, secondary, familial and hereditary cardiac amyloidoses)
  • Systemic diseases (connective tissue disorder; sarcoidosis)
  • Peripartum cardiomyopathy
Both
18 Years to 70 Years
No
Contact: Fulvio Bellocci, MD +390630154187 adellorusso@tin.it
Contact: Antonio Dello Russo, MD +393393971873 adellorusso@tin.it
Italy
 
NCT00127582
GUP02067
Not Provided
Not Provided
Catholic University of the Sacred Heart
Fondazione Telethon
Principal Investigator: Fulvio Bellocci, MD Catholic University of Sacred Heart
Catholic University of the Sacred Heart
May 2005

ICMJE     Data element required by the International Committee of Medical Journal Editors and the World Health Organization ICTRP