Sudden Cardiac Death Stratification in Myotonic Dystrophy Type 1 Patients

NCT ID: NCT03784586

Last Updated: 2021-06-18

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

UNKNOWN

Total Enrollment

70 participants

Study Classification

OBSERVATIONAL

Study Start Date

2012-02-01

Study Completion Date

2023-05-31

Brief Summary

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The aim of the study is to evaluate if the electrophysiological study (EPS) guided therapy, including the prophylactic implantation of implantable cardioverter defibrillator (ICD), in inducible patients, is able to improve survival in comparison with conventional therapy (CONV strategy) in Myotonic Dystrophy type 1 patients with conduction disorders.

Detailed Description

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Trial design:

This is a double-arm prospective observational study.

Patient population:

To be included in the study, DM1 patients had to have at least 18 years old, LVEF \>35% on two-dimensional echocardiography, permanent pacing indication after non-invasive electrocardiographic evaluation or invasive EPS evaluation, according to the current guidelines. The following exclusion criteria were applied: electrolyte imbalance, thyroid disorders, prior cardiac surgery, prior cardiac arrest, prior spontaneous sustained ventricular arrhythmias, absuntion of antiarrhythmic drugs or medication known to affect cardiac conduction. All eligible DM1 patients underwent EPS for programmed ventricular stimulation; those in whom a sustained ventricula arrhythmia was inducible underwent subsequently ICD implantation. Non-inducible DM1 patients did not receive an ICD and underwent pacemaker implantation.

Endpoints:

The primary endpoint was all-cause mortality. Secondary end-points were sudden death (SD), death due to cardiac arrhythmias, ICD interventions and arrhythmic events in implanted patients.

Conditions

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Myotonic Dystrophy 1 Sudden Cardiac Death

Study Design

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Observational Model Type

CASE_CONTROL

Study Time Perspective

PROSPECTIVE

Study Groups

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Positive EPS - ICD

Patients with inducible sustained ventricular tachycardia or ventricular fibrillation at EPS evaluation underwent ICD implantation

No interventions assigned to this group

Negative EPS - PMK

All non inducible patients underwent PMK implantation

No interventions assigned to this group

Eligibility Criteria

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Inclusion Criteria

* MD1 diagnosis genetically confirmed
* LVEF \>35% on two-dimensional echocardiography
* Permanent pacing indication according to the current guidelines

Exclusion Criteria

* Electrolyte imbalance
* Thyroid disorders
* Prior cardiac surgery
* Prior cardiac arrest
* Prior spontaneous sustained ventricular arrhythmias
Minimum Eligible Age

18 Years

Maximum Eligible Age

75 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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University of Campania Luigi Vanvitelli

OTHER

Sponsor Role lead

Responsible Party

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Vincenzo Russo

MD, PhD, Assistant Professor

Responsibility Role PRINCIPAL_INVESTIGATOR

Locations

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Vincenzo Russo

Naples, , Italy

Site Status RECRUITING

Countries

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Italy

Central Contacts

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Vincenzo Russo, MD,PhD

Role: CONTACT

0817064149

Andrea Antonio Papa, MD

Role: CONTACT

0817065158

Other Identifiers

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01_MD1

Identifier Type: -

Identifier Source: org_study_id

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