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

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details. Identifier: NCT04189822
Recruitment Status : Enrolling by invitation
First Posted : December 6, 2019
Last Update Posted : October 20, 2021
Canadian Institutes of Health Research (CIHR)
Information provided by (Responsible Party):
Andrew Krahn, University of British Columbia

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.

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

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Study Type : Observational [Patient Registry]
Estimated Enrollment : 10000 participants
Observational Model: Family-Based
Time Perspective: Prospective
Target Follow-Up Duration: 5 Years
Official Title: Hearts in Rhythm Organization (HiRO)National Registry and Bio Bank: Improving Detection and Treatment of Inherited Heart Rhythm Disorders to Prevent Sudden Death
Actual Study Start Date : August 27, 2019
Estimated Primary Completion Date : December 31, 2025
Estimated Study Completion Date : December 31, 2026

Primary Outcome Measures :
  1. Create a Canadian Research Data base and Bio bank for those affected by inherited heart rhythm conditions. [ Time Frame: November 2019 - June 2025 ]
    To build a data registry and bio bank of 10,000.00 inherited heart rhythm cases

Biospecimen Retention:   Samples With DNA

Blood Samples drawn:

1 X 9 ml EDTA

1 X 10 ml redtop serum tub

Information from the National Library of Medicine

Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the contacts provided below. For general information, Learn About Clinical Studies.

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Ages Eligible for Study:   Child, Adult, Older Adult
Sexes Eligible for Study:   All
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.

Inclusion Criteria:

  • 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


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

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT04189822

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Canada, British Columbia
University of British Columbia/St. Paul's Hospital
Vancouver, British Columbia, Canada, V6E 1M7
Sponsors and Collaborators
University of British Columbia
Canadian Institutes of Health Research (CIHR)
Additional Information:
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Responsible Party: Andrew Krahn, Professor, University of British Columbia Identifier: NCT04189822    
Other Study ID Numbers: H19-01358
First Posted: December 6, 2019    Key Record Dates
Last Update Posted: October 20, 2021
Last Verified: October 2021
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: No

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
Keywords provided by Andrew Krahn, University of British Columbia:
Sudden Cardiac Arrest
Inherited Heart Rhythm Conditions
Additional relevant MeSH terms:
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Heart Arrest
Tachycardia, Ventricular
Cardiomyopathy, Hypertrophic
Long QT Syndrome
Death, Sudden, Cardiac
Arrhythmogenic Right Ventricular Dysplasia
Pathologic Processes
Heart Diseases
Cardiovascular Diseases
Arrhythmias, Cardiac
Cardiac Conduction System Disease
Aortic Stenosis, Subvalvular
Aortic Valve Stenosis
Aortic Valve Disease
Heart Valve Diseases
Heart Defects, Congenital
Cardiovascular Abnormalities
Congenital Abnormalities
Death, Sudden