National ARVC Data Registry and Bio Bank

NCT01804699 | Completed

• 1 other identifier: H12-03189
• Study Type: observational
• Target: 1,500
• Locations: 1 country
• Sites: 16

Brief Summary

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is an inherited condition that may cause life threatening irregular heart rhythms that often manifest as unexpected cardiac arrest or sudden death in early adulthood. The condition is difficult to diagnose and often is not noticed until a family member suffers a cardiac arrest or death. The Canadian National ARVC registry will collect data from Inherited Heart Rhythm Clinics across Canada. STUDY OBJECTIVES: Primary:

1. 1.To determine the natural history of ARVC (short/intermediate term), including risk of symptomatic arrhythmias and sudden death, for patients with the phenotype and those gene positive patients without phenotype evidence of disease.
2. 2.To understand risk factors for sudden death/appropriate ICD use in ARVC, including test characteristics/performance and their relationship to outcomes (ECG, Holter, signal averaged ECG, loop recorders, imaging, voltage mapping, T wave alternans, cardiac biopsy and biomarkers).
3. 3.To establish a phenotype genotype correlation, including comparison of patients with disease causing mutations, variants of unknown significance (VUS) and Task Force Criteria (TFC) positive, gene negative patients

Conditions

Arrhythmogenic Right Ventricular Cardiomyopathy

Keywords

ARVC, Cardiomyopathy

Outcome Measures

Primary Outcomes (1)

• Natural History of ARVC

  To determine the natural history of ARVC (short/intermediate term), including risk of symptomatic arrhythmias and sudden death, for patients with the phenotype and those gene positive patients without phenotype evidence of disease.

  Three years

Secondary Outcomes (1)

• Risk Factors and Sudden Death

  Three Years

Other Outcomes (1)

• Establishing a phenotype genotype correlation

  Three Years

Study Arms (2)

Proband

Probands - Participants diagnosed with ARVC according to the 2010 Task Force Criteria

Family

First Degree Family member (blood related mother, father, sister, brother, child) of the proband

Eligibility Criteria

• Age: 2 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

Patients will be identified from centers that form the Canadian Genetics Heart Rhythm (CGHR) Network of Inherited Heart Rhythm Clinics across Canada 25 (http://www.heartrhythmresearch.ca/).

You may not qualify if:

• Known condition that mimics ARVC - sarcoidosis (biopsy proven or with lung involvement), familial dilated cardiomyopathy not compatible with an ARVC variant, hypertrophic cardiomyopathy
• Known inherited condition that predisposes to sudden death - Long or Short QT Syndrome, Catecholaminergic Polymorphic Ventricular Tachycardia and Brugada Syndrome
• Age \< 2 years
• Life expectancy less than 1 year
• Unable and/or unwilling to provide informed consent

Sponsors & Collaborators

• University of British Columbia: lead
• Population Health Research Institute: collaborator
• Medtronic: collaborator
• Boston Scientific Corporation: collaborator
• Canadian Institutes of Health Research (CIHR): collaborator

Study Sites (16)

Libin Cardiovascular Institute of Alberta, University of Calgary

Calgary, Alberta, Canada

Location

BC Children's Hospital

Vancouver, British Columbia, Canada

Location

University of British Columbia, St. Paul's Hospital

Vancouver, British Columbia, Canada

Location

University of Victoria, Victoria Jubilee Hospital

Victoria, British Columbia, Canada

Location

St. Boniface Hospital

Winnipeg, Manitoba, Canada

Location

Queen Elizabeth II, Halifax Infirmary

Halifax, Nova Scotia, Canada

Location

McMaster University, Faculty of Health Sciences

Hamilton, Ontario, Canada

Location

Lawson Health Research Institute

London, Ontario, Canada

Location

Ottawa Heart Institute

Ottawa, Ontario, Canada

Location

St. Michael's Hospital

Toronto, Ontario, Canada

Location

The Hospital for Sick Children

Toronto, Ontario, Canada

Location

Toronto General Hospital

Toronto, Ontario, Canada

Location

McGill University Health Centre

Montreal, Quebec, Canada

Location

Montreal Heart Institute

Montreal, Quebec, Canada

Location

St. Justine Hospital

Montreal, Quebec, Canada

Location

Institut Universitaire de Cardiologie, Université Laval

Québec, Quebec, Canada

Location

Biospecimen

Retention: SAMPLES WITH DNA

If the participant consents to the biobanking arm of the study an additional blood sample (Please see the ARVC Lab Manual for details) will be collected at baseline and stored for subsequent analysis. These analyses may include measurement of serum Troponin T, NT-pro-BNP, C-reactive protein and other biomarkers to determine their association with the progression of disease; both in terms of cardiac structure (i.e. change in right ventricular volume) and electrical substrate (i.e. number of ICD shocks) over time.

MeSH Terms

Conditions

Arrhythmogenic Right Ventricular Dysplasia; Cardiomyopathies

Condition Hierarchy (Ancestors)

Heart Defects, Congenital; Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Andrew D Krahn, MD

  University of British Columbia, Dept of Medicine, Head of Cardiology

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: FAMILY BASED
• Time Perspective: PROSPECTIVE
• Target Duration: 3 Years
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Professor

Study Record Dates

• First Submitted: March 1, 2013
• First Posted: March 5, 2013
• Study Start: January 1, 2013
• Primary Completion: August 1, 2020
• Study Completion: August 1, 2020
• Last Updated: November 16, 2020

Record last verified: 2020-11

Data Sharing

• IPD Sharing: Will not share

Locations

CA (16)