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RAMYD Study - Evaluation of Arrhythmic Risk in Myotonic Dystrophy

Primary Purpose

Myotonic Dystrophy, Sudden Cardiac Death

Status
Unknown status
Phase
Phase 3
Locations
Italy
Study Type
Interventional
Intervention
Electrophysiological study
pacemaker (PM) implant, internal cardiac defibrillator (ICD) implant, loop-recorder implant
Sponsored by
Catholic University of the Sacred Heart
About
Eligibility
Locations
Outcomes
Full info

About this trial

This is an interventional treatment trial for Myotonic Dystrophy focused on measuring Myotonic dystrophy type 1, Sudden cardiac death, Ventricular tachyarrhythmias, Bradyarrhythmias

Eligibility Criteria

18 Years - 70 Years (Adult, Older Adult)All SexesDoes not accept healthy volunteers

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

Sites / Locations

  • Catholic University of Sacred HeartRecruiting

Outcomes

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

Full Information

First Posted
August 4, 2005
Last Updated
August 24, 2005
Sponsor
Catholic University of the Sacred Heart
Collaborators
Fondazione Telethon
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1. Study Identification

Unique Protocol Identification Number
NCT00127582
Brief Title
RAMYD Study - Evaluation of Arrhythmic Risk in Myotonic Dystrophy
Official Title
Evaluation of Arrhythmic Risk in Myotonic Dystrophy Type I (DM 1)
Study Type
Interventional

2. Study Status

Record Verification Date
May 2005
Overall Recruitment Status
Unknown status
Study Start Date
January 2003 (undefined)
Primary Completion Date
undefined (undefined)
Study Completion Date
undefined (undefined)

3. Sponsor/Collaborators

Name of the Sponsor
Catholic University of the Sacred Heart
Collaborators
Fondazione Telethon

4. Oversight

5. Study Description

Brief Summary
This is a prospective multicentric Italian study to evaluate the arrhythmic risk in myotonic dystrophy type 1.
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: Clinical-genetic evaluation; Non invasive and invasive diagnostic cardiac procedures; The use of devices for diagnostic and therapeutic follow-up.

6. Conditions and Keywords

Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Myotonic Dystrophy, Sudden Cardiac Death
Keywords
Myotonic dystrophy type 1, Sudden cardiac death, Ventricular tachyarrhythmias, Bradyarrhythmias

7. Study Design

Primary Purpose
Treatment
Study Phase
Phase 3
Interventional Study Model
Single Group Assignment
Masking
None (Open Label)
Allocation
Non-Randomized
Enrollment
537 (false)

8. Arms, Groups, and Interventions

Intervention Type
Procedure
Intervention Name(s)
Electrophysiological study
Intervention Type
Device
Intervention Name(s)
pacemaker (PM) implant, internal cardiac defibrillator (ICD) implant, loop-recorder implant
Primary Outcome Measure Information:
Title
Evaluate incidence of: major cardiac events (sudden death
Title
resuscitated cardiac arrest
Title
ventricular fibrillation
Title
sustained ventricular tachycardia
Title
sinoatrial and atrioventricular [AV] blocks)
Secondary Outcome Measure Information:
Title
Evaluate with diagnostic non-invasive (standard electrocardiogram [ECG]
Title
24-hour monitoring ECG
Title
signal-averaged ECG
Title
echocardiography) and invasive procedures (electrophysiology study [EPS] and implantable loop recorders) the risk to develop cardiac arrhythmias in DM patients

10. Eligibility

Sex
All
Minimum Age & Unit of Time
18 Years
Maximum Age & Unit of Time
70 Years
Accepts Healthy Volunteers
No
Eligibility 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
Central Contact Person:
First Name & Middle Initial & Last Name or Official Title & Degree
Fulvio Bellocci, MD
Phone
+390630154187
Email
adellorusso@tin.it
First Name & Middle Initial & Last Name or Official Title & Degree
Antonio Dello Russo, MD
Phone
+393393971873
Email
adellorusso@tin.it
Overall Study Officials:
First Name & Middle Initial & Last Name & Degree
Fulvio Bellocci, MD
Organizational Affiliation
Catholic University of Sacred Heart
Official's Role
Principal Investigator
Facility Information:
Facility Name
Catholic University of Sacred Heart
City
Rome
ZIP/Postal Code
00168
Country
Italy
Individual Site Status
Recruiting
Facility Contact:
First Name & Middle Initial & Last Name & Degree
Fulvio Bellocci, MD
Phone
+390630154187
Email
adellorusso@tin.it
First Name & Middle Initial & Last Name & Degree
Fulvio Bellocci, MD

12. IPD Sharing Statement

Citations:
PubMed Identifier
12433913
Citation
Pelargonio G, Dello Russo A, Sanna T, De Martino G, Bellocci F. Myotonic dystrophy and the heart. Heart. 2002 Dec;88(6):665-70. doi: 10.1136/heart.88.6.665. No abstract available.
Results Reference
background
PubMed Identifier
19708226
Citation
Dello Russo A, Mangiola F, Della Bella P, Nigro G, Melacini P, Bongiorni MG, Tondo C, Calo L, Messano L, Pace M, Pelargonio G, Casella M, Sanna T, Silvestri G, Modoni A, Zachara E, Moltrasio M, Morandi L, Nigro G, Politano L, Palladino A, Bellocci F. Risk of arrhythmias in myotonic dystrophy: trial design of the RAMYD study. J Cardiovasc Med (Hagerstown). 2009 Jan;10(1):51-8. doi: 10.2459/jcm.0b013e328319bd2c.
Results Reference
derived

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RAMYD Study - Evaluation of Arrhythmic Risk in Myotonic Dystrophy

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