Sudden Cardiac Death Stratification in Myotonic Dystrophy Type 1 Patients

• Conditions: Sudden Cardiac Death; Myotonic Dystrophy 1

• Registration Number: NCT03784586
• Lead Sponsor: University of Campania "Luigi Vanvitelli"

Brief Summary

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

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.

Study Timeline

• Study Start: 2012-02-01
• Study First Submitted: 2018-12-17
• Study First Submitted QC: 2018-12-21
• Study First Posted: 2018-12-24
• Status Verified: 2021-06
• Last Update Submitted: 2021-06-13
• Last Update Posted: 2021-06-18
• Primary Completion: 2022-06-30
• Study Completion: 2023-05-31

Recruitment & Eligibility

• Status: UNKNOWN
• Sex: All
• Target Recruitment: 70

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

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Sudden Cardiac Death	24 months

Secondary Outcome Measures

Name	Time	Method
All-cause mortality	24 months

Trial Locations

Locations (1)

Vincenzo Russo; 🇮🇹 Naples, Italy