Arrhythmogenic Right Ventricular Cardiomyopathy.
Andrew D Krahn, Arthur A M Wilde, Hugh Calkins...
https://pubmed.ncbi.nlm.nih.gov/35450611Actively Recruiting
Phase 2
Age: 18Years +
All Genders
ID06174220
Targeted Therapy With Glycogen Synthase Kinase-3 Inhibition for Arrhythmogenic Cardiomyopathy
Led by Hamilton Health Sciences Corporation · Updated on 2026-03-12
120
Participants Needed
17
Research Sites
21 weeks
Total Duration
On this page
Sponsors
H
Hamilton Health Sciences Corporation
Lead Sponsor
C
Canadian Institutes of Health Research (CIHR)
Collaborating Sponsor
AI-Summary
What this Trial Is About
Researchers are investigating the potential benefits of tideglusib, a drug that inhibits glycogen synthase kinase-3 beta, in patients with arrhythmogenic cardiomyopathy (ACM), a genetic heart disease that increases the risk of dangerous heart rhythms and sudden cardiac death. This phase 2 study focuses on patients with specific genetic variants linked to ACM and aims to better understand how tideglusib might affect the disease. The trial is randomized, double-blind, and placebo-controlled to ensure reliable results. Participants are randomly assigned to receive either tideglusib 1 gram by mouth daily or a matching placebo for six months. The study compares the effects of this treatment on heart rhythm irregularities and heart function. The treatment phase is followed by assessments to monitor changes in premature ventricular contractions and other heart-related outcomes. Throughout the study, participants will undergo evaluations including heart rhythm monitoring using Holter devices, assessments of ventricular strain, and tracking of implantable cardioverter-defibrillator (ICD) therapies. Researchers will measure the number of premature ventricular contractions and episodes of sustained ventricular tachycardia before and after six months of treatment. Safety and adherence will be monitored during this period, and the total participation lasts approximately six months.
CONDITIONS
Brief Title
Targeted Therapy With Glycogen Synthase Kinase-3 Inhibition for Arrhythmogenic Cardiomyopathy
Who Can Participate
Age: 18Years +
All Genders
Eligibility Criteria
You may qualify if you...
- Have a pathogenic or likely pathogenic desmosomal gene variant (PKP2, DSG2, DSC2, DSP, or JUP) or the TMEM43-p.S358L variant
- JUP gene carriers must be homozygous or compound heterozygous
- Have an average of at least 500 premature ventricular contractions in 24 hours on a baseline 7-day Holter monitor
- Have a clinical diagnosis of arrhythmogenic cardiomyopathy or known genetic carrier status for at least 6 months before screening
- Be 18 years of age or older
You will not qualify if you...
- Have NYHA class IV heart failure
- Have ventricular scar caused by coronary artery disease
- Changed dose or started/stopped Class I or III anti-arrhythmic drugs within 3 months before screening
- Have any harmful chronic liver disease
- Have ALT blood levels more than twice the normal upper limit at screening
- Have total bilirubin levels above normal limits at screening (except if Gilbert's syndrome, then more than twice the normal limit)
- Have a history of alcohol or illicit substance use disorders
- Regularly use strong CYP3A4 inhibitors such as clarithromycin, ketoconazole, or ritonavir
- Have serum creatinine above 150 micromoles/L or creatinine clearance 60 mL/min or less at screening
- Are pregnant or, if female of childbearing potential, do not use highly effective contraception
- Are male and have sexual relations with females of childbearing potential without acceptable contraception if not surgically sterile
- Are unwilling to give informed consent or follow study procedures
- Are allergic to tideglusib or any of its ingredients, including strawberry allergy
- Use drugs metabolized by CYP3A4 with a narrow therapeutic range, like warfarin or digoxin concurrently
AI-Screening
AI-Powered Screening
Complete this quick 3-step screening to check your eligibility
1
2
3
Your Study Journey
Screening
Duration - 2 to 4 weeks
Participants are screened for eligibility to participate in the trial.
1 visit (in-person)
Treatment
Duration - 6 months
Participants receive either Tideglusib or a matching placebo daily to evaluate the effects on arrhythmogenic cardiomyopathy.
Regular visits to monitor treatment effects and safety
Trial Site Locations
Total: 17 locations
1
University of Calgary
Calgary, Alberta, Canada, T2N 2T9
Actively Recruiting
2
University of British Columbia
Vancouver, British Columbia, Canada, V6Z 1Y6
Actively Recruiting
3
Victoria Cardiac Arrhythmia Trials Inc.
Victoria, British Columbia, Canada, V8Z 0B9
Actively Recruiting
4
NL Health Services
St. John's, Newfoundland and Labrador, Canada, A1B 3V6
Actively Recruiting
5
Nova Scotia Health
Halifax, Nova Scotia, Canada, B3H 2Y9
Actively Recruiting
6
Hamilton General Hospital
Hamilton, Ontario, Canada, L8L 2X2
Actively Recruiting
7
Kingston General Hospital
Kingston, Ontario, Canada, K7L 2V7
Not Yet Recruiting
8
London Health Sciences Centre
London, Ontario, Canada, N6A 5A5
Actively Recruiting
9
Newmarket Electrophysiologist Research Group 'NERG'
Newmarket, Ontario, Canada, L3Y 2P9
Not Yet Recruiting
10
University of Ottawa Heart Institute
Ottawa, Ontario, Canada, K1Y 4W7
Not Yet Recruiting
11
Heart Health Institute Research Inc
Scarborough Village, Ontario, Canada, M1E 4B9
Actively Recruiting
12
Sunnybrook Health Sciences Centre
Toronto, Ontario, Canada, M4N 3M5
Not Yet Recruiting
13
St. Michael's Hospital
Toronto, Ontario, Canada, M5B 1W8
Not Yet Recruiting
14
Montreal Heart Institute
Montreal, Quebec, Canada, H1T 1C8
Actively Recruiting
15
McGill University Health Centre
Montreal, Quebec, Canada, H4A 3J1
Not Yet Recruiting
16
Hopital du Sacré-Coeur de Montréal
Montreal, Quebec, Canada, H4J 1C5
Not Yet Recruiting
17
University Institute of Cardiology and Pneumology of Quebec
Québec, Quebec, Canada, G1V 4G5
Actively Recruiting
Loading map...
Research Team
J
Jason Roberts, MD MAS
M
Maha Mushtaha
How is the study designed?
Study Type
INTERVENTIONAL
Masking
QUADRUPLE
Allocation
RANDOMIZED
Model
PARALLEL
Primary Purpose
TREATMENT
Number of Arms
2
Similar Trials
Frequently Asked Questions
Have more questions? Get in touch with our team for quick support
Not the Right Trial for You?
Explore thousands of other clinical trials that might be a better match.
Sign up to get personalized trial recommendations delivered to your inbox.
Already have an account? Log in here
Published Research Related To This Trial
Identification of a new modulator of the intercalated disc in a zebrafish model of arrhythmogenic cardiomyopathy.
Angeliki Asimaki, Sudhir Kapoor, Eva Plovie...
https://pubmed.ncbi.nlm.nih.gov/24920660Central role for GSK3β in the pathogenesis of arrhythmogenic cardiomyopathy.
Stephen P Chelko, Angeliki Asimaki, Peter Andersen...
https://pubmed.ncbi.nlm.nih.gov/27170944Ankyrin-B dysfunction predisposes to arrhythmogenic cardiomyopathy and is amenable to therapy.
Jason D Roberts, Nathaniel P Murphy, Robert M Hamilton...
https://pubmed.ncbi.nlm.nih.gov/31264976Severe Cardiac Dysfunction and Death Caused by Arrhythmogenic Right Ventricular Cardiomyopathy Type 5 Are Improved by Inhibition of Glycogen Synthase Kinase-3β.
Laura Padrón-Barthe, María Villalba-Orero, Jesús M Gómez-Salinero...
https://pubmed.ncbi.nlm.nih.gov/31567019