Sudden Cardiac Death Stratification in Myotonic Dystrophy Type 1 Patients

Study Overview
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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.

Official Title
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The Electrophysiological Study Guided ICD Strategy in Prevention of Arrhythmic Cardiac Death in Myotonic Dystrophy Type 1 Patients With Conduction System Disease (ACADEMY 1 Study)

Conditions
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Myotonic Dystrophy 1, Sudden Cardiac Death

Participation Criteria
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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

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 75 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Positive EPS - ICD<br>Patients with inducible sustained ventricular tachycardia or ventricular fibrillation at EPS evaluation underwent ICD implantation | |
| Negative EPS - PMK<br>All non inducible patients underwent PMK implantation | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Sudden Cardiac Death |  | 24 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| All-cause mortality |  | 24 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Arrhythmias, electrophisiological study, primary prevention