Sudden Cardiac Death Stratification in Myotonic Dystrophy Type 1 Patients (ACADEMY 1)

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)

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.

Study Overview

• Status: Recruiting
• Conditions: Sudden Cardiac Death; Myotonic Dystrophy 1

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 Type: Observational
• Enrollment (Anticipated): 70

Contacts and Locations

• Study Contact: Name: Vincenzo Russo, MD,PhD; Phone Number: 0817064149; Email: v.p.russo@libero.it
• Study Contact Backup: Name: Andrea Antonio Papa, MD; Phone Number: 0817065158; Email: andreaantoniopapa@libero.it

Study Locations

• Italy
  • Naples, Italy, 80136
    • Recruiting
    • Vincenzo Russo

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 75 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

MD1 patients with permanent pacing indication and needed of sudden cardiac death screening.

Description

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 Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort
Positive EPS - ICD; Patients with inducible sustained ventricular tachycardia or ventricular fibrillation at EPS evaluation underwent ICD implantation
Negative EPS - PMK; All non inducible patients underwent PMK implantation

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Sudden Cardiac Death	24 months

Secondary Outcome Measures

Outcome Measure	Time Frame
All-cause mortality	24 months

Collaborators and Investigators

• Sponsor: University of Campania "Luigi Vanvitelli"

Study record dates

Study Major Dates

• Study Start (Actual): February 1, 2012
• Primary Completion (Anticipated): June 30, 2022
• Study Completion (Anticipated): May 31, 2023

Study Registration Dates

• First Submitted: December 17, 2018
• First Submitted That Met QC Criteria: December 21, 2018
• First Posted (Actual): December 24, 2018

Study Record Updates

• Last Update Posted (Actual): June 18, 2021
• Last Update Submitted That Met QC Criteria: June 13, 2021
• Last Verified: June 1, 2021

More Information

Terms related to this study

• Keywords: primary prevention; Arrhythmias; electrophisiological study
• Additional Relevant MeSH Terms: Pathologic Processes; Heart Diseases; Cardiovascular Diseases; Nervous System Diseases; Genetic Diseases, Inborn; Musculoskeletal Diseases; Muscular Diseases; Neuromuscular Diseases; Neurodegenerative Diseases; Death, Sudden; Muscular Disorders, Atrophic; Heredodegenerative Disorders, Nervous System; Muscular Dystrophies; Myotonic Disorders; Heart Arrest; Death; Myotonic Dystrophy; Death, Sudden, Cardiac
• Other Study ID Numbers: 01_MD1

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No