Hearts in Rhythm Organization (HiRO)National Registry and Bio Bank

NCT04189822 | Enrolling By Invitation

• 1 other identifier: H19-01358
• Study Type: observational
• Target: 10,000
• Locations: 1 country
• Sites: 1

Brief Summary

The Hearts in Rhythm Organization (HiRO) is a national network of Canadian researchers/clinicians, working towards a better understanding of the rare genetic causes of sudden cardiac death (SCD). Canadian adult and pediatric electrophysiology centres across Canada work together to gather data and bio sample in a national data registry and bio bank hoping to improve the detection and treatment of inherited heart rhythm disorders to prevent sudden death.

Conditions

Sudden Cardiac Arrest; Sudden Arrhythmic Death Syndrome; Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC); Long QT Syndrome (LQTS); Hypertrophic Cardiomyopathy (HCM); Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)

Keywords

Sudden Cardiac Arrest; Inherited Heart Rhythm Conditions; Cardiogenetics

Outcome Measures

Primary Outcomes (1)

• Create a Canadian Research Data base and Bio bank for those affected by inherited heart rhythm conditions.

  To build a data registry and bio bank of 10,000.00 inherited heart rhythm cases

  November 2019 - June 2025

Eligibility Criteria

• Sex: all
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

The registry will include all willing participants seen as patients in the HiRO network that are evaluated for familial risk of sudden death. The registry will include, inheritable heart rhythm conditions including but not restricted to; Brugada Syndrome, Long QT syndrome, hypertrophic cardiomyopathy (HCM), catecholaminergic polymorphic ventricular tachycardia (CPVT), and arrhythmogenic right ventricular cardiomyopathy (ARVC) and uncommon variants of ARVC that are listed fully in the inclusion criteria section of this protocol as well as first degree family members.

You may qualify if:

• All Canadian patients referred for cardiac investigations related to inherited heart rhythm (IHR) conditions will be invited to participate if they meet the following criteria:
• They understand the registry/bio bank purpose, potential risks and willingly sign consent
• Recognized genetic syndromes; Long QT syndrome (LQT), Short QT Syndrome (SQT), catecholaminergic polymorphic ventricular tachycardia (CPVT), Brugada (BrS), Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), Familial Cardiac Conduction Disease (FCCD).
• Deceased cases of SCD, suspicious for an inherited heart rhythm condition. Included with signed consent from next of kin (NOK).
• IHR patients referred for risk of SCD enrolled (includes first degree and second degree relatives) SCD syndromes seen in heart rhythm clinics; Unexplained cardiac arrest (UCA), Early Repolarization (ER), Idiopathic Ventricular Fibrillation (IVF), Short Coupled IVF (SCIF), Polymorphic Ventricular Tachycardia Not Otherwise Diagnosed (PMVT, NYD), Sudden Arrhythmic Death Syndromes (SADS) that are SCD cases with negative autopsy results.
• Mendelian cardiomyopathies (hypertrophic cardiomyopathy (HCM), Mendelian Dilated Cardiomyopathy (DCM) including Lamin and Phosopholambin (LMNA, \& PLN), and Left Ventricular Non -Compaction (LVNC).
• \*\*Must have either a probable or definite clinical diagnosis, first degree relative (FDR) with a known diagnosis, gene carrier (disease causing or likely disease causing by American College of Medical Genetics (ACMG 2015), or may be second degree relative (SDR) with inability to screen intervening relative
• Carriers of a pathogenic or likely-pathogenic variant for an inherited arrhythmia or cardiomyopathy related gene, not otherwise fitting inherited or cardiomyopathy diagnostic criteria

You may not qualify if:

• Patients referred for cardiac investigations related to inherited heart rhythm conditions will be excluded from participating if they meet the following criteria:
• Unwilling or are unable to provide informed consent
• Known sarcoidosis
• Mitral valve Prolapse unless unexplained cardiac arrest or syncope with documented PMVT
• Heart Failure/Non-Familial Dilated Cardiomyopathy DCM without a positive family history of affected FDRs or SDRs
• Aortopathies including Marfan Syndrome, Ehlers Danlos, Familial Thoracic Aortic Aneurysm and Dissection
• Neuromuscular disease
• Familial hypercholesterolemia

Sponsors & Collaborators

• University of British Columbia: lead
• Canadian Institutes of Health Research (CIHR): collaborator

Study Sites (1)

University of British Columbia/St. Paul's Hospital

Vancouver, British Columbia, V6E 1M7, Canada

Location

Related Publications (3)

• Mellor G, Laksman ZWM, Tadros R, Roberts JD, Gerull B, Simpson CS, Klein GJ, Champagne J, Talajic M, Gardner M, Steinberg C, Arbour L, Birnie DH, Angaran P, Leather R, Sanatani S, Chauhan VS, Seifer C, Healey JS, Krahn AD. Genetic Testing in the Evaluation of Unexplained Cardiac Arrest: From the CASPER (Cardiac Arrest Survivors With Preserved Ejection Fraction Registry). Circ Cardiovasc Genet. 2017 Jun;10(3):e001686. doi: 10.1161/CIRCGENETICS.116.001686.

  PMID: 28600387 BACKGROUND

• Krahn AD, Healey JS, Chauhan V, Birnie DH, Simpson CS, Champagne J, Gardner M, Sanatani S, Exner DV, Klein GJ, Yee R, Skanes AC, Gula LJ, Gollob MH. Systematic assessment of patients with unexplained cardiac arrest: Cardiac Arrest Survivors With Preserved Ejection Fraction Registry (CASPER). Circulation. 2009 Jul 28;120(4):278-85. doi: 10.1161/CIRCULATIONAHA.109.853143. Epub 2009 Jul 13.

  PMID: 19597050 BACKGROUND

• Herman AR, Cheung C, Gerull B, Simpson CS, Birnie DH, Klein GJ, Champagne J, Healey JS, Gibbs K, Talajic M, Gardner M, Bennett MT, Steinberg C, Janzen M, Gollob MH, Angaran P, Yee R, Leather R, Chakrabarti S, Sanatani S, Chauhan VS, Krahn AD. Outcome of Apparently Unexplained Cardiac Arrest: Results From Investigation and Follow-Up of the Prospective Cardiac Arrest Survivors With Preserved Ejection Fraction Registry. Circ Arrhythm Electrophysiol. 2016 Jan;9(1):e003619. doi: 10.1161/CIRCEP.115.003619.

  PMID: 26783233 BACKGROUND

Related Links

• HiRO website

Biospecimen

Retention: SAMPLES WITH DNA

Blood Samples drawn: 1 X 9 ml EDTA 1 X 10 ml redtop serum tub Saliva may be collected as an alternative to blood

MeSH Terms

Conditions

Death, Sudden, Cardiac; Arrhythmogenic Right Ventricular Dysplasia; Long QT Syndrome; Cardiomyopathy, Hypertrophic; Polymorphic Catecholaminergic Ventricular Tachycardia

Condition Hierarchy (Ancestors)

Heart Arrest; Heart Diseases; Cardiovascular Diseases; Death, Sudden; Death; Pathologic Processes; Pathological Conditions, Signs and Symptoms; Heart Defects, Congenital; Cardiovascular Abnormalities; Cardiomyopathies; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Arrhythmias, Cardiac; Cardiac Conduction System Disease; Aortic Stenosis, Subvalvular; Aortic Valve Stenosis; Aortic Valve Disease; Heart Valve Diseases; Tachycardia, Ventricular; Tachycardia

Study Design

• Study Type: observational
• Observational Model: FAMILY BASED
• Time Perspective: PROSPECTIVE
• Target Duration: 5 Years
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Professor

Study Record Dates

• First Submitted: November 18, 2019
• First Posted: December 6, 2019
• Study Start: August 27, 2019
• Primary Completion: December 31, 2025
• Study Completion (Estimated): December 31, 2026
• Last Updated: November 3, 2022

Record last verified: 2022-10

Data Sharing

• IPD Sharing: Will not share

Locations

CA (1)