Trial record 5 of 17 for:    "Myotonic dystrophy"

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
  Purpose

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


Condition Intervention Phase
Myotonic Dystrophy
Sudden Cardiac Death
Procedure: Electrophysiological study
Device: pacemaker (PM) implant, internal cardiac defibrillator (ICD) implant, loop-recorder implant
Phase 3

Study Type: Interventional
Study Design: Allocation: Non-Randomized
Endpoint Classification: Safety Study
Intervention Model: Single Group Assignment
Masking: Open Label
Primary Purpose: Treatment
Official Title: Evaluation of Arrhythmic Risk in Myotonic Dystrophy Type I (DM 1)

Resource links provided by NLM:


Further study details as provided by Catholic University of the Sacred Heart:

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

Secondary Outcome Measures:
  • 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

Estimated Enrollment: 537
Study Start Date: January 2003
Detailed Description:

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.
  Eligibility

Ages Eligible for Study:   18 Years to 70 Years
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Criteria

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
  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 ClinicalTrials.gov identifier: NCT00127582

Contacts
Contact: Fulvio Bellocci, MD +390630154187 adellorusso@tin.it
Contact: Antonio Dello Russo, MD +393393971873 adellorusso@tin.it

Locations
Italy
Catholic University of Sacred Heart Recruiting
Rome, Italy, 00168
Contact: Fulvio Bellocci, MD    +390630154187    adellorusso@tin.it   
Principal Investigator: Fulvio Bellocci, MD         
Sponsors and Collaborators
Catholic University of the Sacred Heart
Fondazione Telethon
Investigators
Principal Investigator: Fulvio Bellocci, MD Catholic University of Sacred Heart
  More Information

Publications:
Additional publications automatically indexed to this study by ClinicalTrials.gov Identifier (NCT Number):
ClinicalTrials.gov Identifier: NCT00127582     History of Changes
Other Study ID Numbers: GUP02067
Study First Received: August 4, 2005
Last Updated: August 24, 2005
Health Authority: Italy: Ministry of Health

Keywords provided by Catholic University of the Sacred Heart:
Myotonic dystrophy type 1
Sudden cardiac death
Ventricular tachyarrhythmias
Bradyarrhythmias

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

ClinicalTrials.gov processed this record on August 28, 2014