NCT00127582

Brief Summary

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

Trial Health

87
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
537

participants targeted

Target at P75+ for not_applicable

Timeline
Completed

Started Jan 2003

Longer than P75 for not_applicable

Geographic Reach
1 country

1 active site

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Start

First participant enrolled

January 1, 2003

Completed
2.6 years until next milestone

First Submitted

Initial submission to the registry

August 4, 2005

Completed
4 days until next milestone

First Posted

Study publicly available on registry

August 8, 2005

Completed
4.3 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

December 1, 2009

Completed
1 year until next milestone

Study Completion

Last participant's last visit for all outcomes

December 1, 2010

Completed
Last Updated

March 19, 2026

Status Verified

March 1, 2026

Enrollment Period

6.9 years

First QC Date

August 4, 2005

Last Update Submit

March 17, 2026

Conditions

Sudden Cardiac DeathMyotonic Dystrophy

Keywords

Myotonic dystrophy type 1Sudden cardiac deathVentricular tachyarrhythmiasBradyarrhythmias

Outcome Measures

Primary Outcomes (2)

  • Incidence of major cardiac events (sudden death)

    To assess the 2-year cumulative incidence of sudden death in patients affected by Myotonic dystrophy type 1.

    2 years

  • Resuscitated cardiac arrest

    To evaluate the value of noninvasive and invasive findings as predictive factors for resuscitated cardiac arrest.

    2 years

Study Arms (1)

Patient affected by myotonic dystrophy type I (MD1)

EXPERIMENTAL

Cardiac involvement represents a major problem in the clinical management of patient affected by myotonic dystrophy type I (MD1), for whom cardiac complications represent one of the primary causes of premature death. 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.

Procedure: Electrophysiological studyDevice: pacemaker , implantable cardiac defibrillator or loop-recorder implant

Interventions

pacemaker , implantable cardiac defibrillator or loop-recorder implantDEVICE
Patient affected by myotonic dystrophy type I (MD1)
Electrophysiological studyPROCEDURE
Patient affected by myotonic dystrophy type I (MD1)

Eligibility Criteria

Age18 Years - 70 Years
Sexall
Healthy VolunteersNo
Age GroupsAdult (18-64), Older Adult (65+)

You may qualify if:

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

You may not qualify if:

  • 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

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (1)

Catholic University of Sacred Heart

Rome, 00168, Italy

Location

Related Publications (2)

  • 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.

    PMID: 12433913BACKGROUND

  • 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.

    PMID: 19708226DERIVED

MeSH Terms

Conditions

Myotonic DystrophyDeath, Sudden, CardiacTachycardia, VentricularBradycardia

Condition Hierarchy (Ancestors)

Muscular DystrophiesMuscular Disorders, AtrophicMuscular DiseasesMusculoskeletal DiseasesMyotonic DisordersHeredodegenerative Disorders, Nervous SystemNeurodegenerative DiseasesNervous System DiseasesNeuromuscular DiseasesGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesHeart ArrestHeart DiseasesCardiovascular DiseasesDeath, SuddenDeathPathologic ProcessesPathological Conditions, Signs and SymptomsTachycardiaArrhythmias, CardiacCardiac Conduction System Disease

Study Officials

  • Fulvio Bellocci, MD

    Catholic University of Sacred Heart

    PRINCIPAL INVESTIGATOR

Study Design

Study Type
interventional
Phase
not applicable
Allocation
NA
Masking
NONE
Purpose
DIAGNOSTIC
Intervention Model
SINGLE GROUP
Sponsor Type
OTHER
Responsible Party
SPONSOR

Study Record Dates

First Submitted

August 4, 2005

First Posted

August 8, 2005

Study Start

January 1, 2003

Primary Completion

December 1, 2009

Study Completion

December 1, 2010

Last Updated

March 19, 2026

Record last verified: 2026-03

Locations

IT(1)