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HiRO Registry

HiRO

Hearts in Rhythm Organization (HiRO)National Registry and Bio Bank: Improving Detection and Treatment of Inherited Heart Rhythm Disorders to Prevent Sudden Death

Lead PI & Sponsor

Dr. Andrew Krahn

Local PI

Dr. Habib Khan

Research Staff

Megan Smith, Sidney Trinh

Objective

To create a Canadian research database and bio bank, designed to collect healthcare information and samples on patients being investigated and treated for inherited heart rhythm conditions. This will provide a means to study inherited heart rhythm conditions that will provide an accurate picture of this population in Canada.

Target Number of Patients

2500

Currently Enrolled

1185

Primary Outcomes

  • To engage 90+% of Inherited Heart Rhythm patients in clinical/translational research
  • To develop a 10,000 case registry of consenting participants at genetic risk for sudden death and their first-degree relatives

Secondary Outcomes

  • To develop new care pathways including case finding, risk assessment algorithms, improved guidance for prevention and treatment of inherited arrhythmias, as well as related knowledge translation
  • To develop a patient education curriculum and educational materials/tool
  • To develop a system/tool to measure clinical outcomes
  • To create a national research data base for inherited heart rhythm conditions
  • To improve the efficiency and accessibility of research participation for all Canadians willing to be included in the registry and store their bio sample
  • To improve the care for Inherited Heart Rhythm patients
  • To provide optional bio banking of DNA and Serum for future research

Inclusion Criteria

  • 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).
  • Carriers of a pathogenic or likely-pathogenic variant for an inherited arrhythmia or cardiomyopathy related gene, not otherwise fitting inherited or cardiomyopathy diagnostic criteria

Exclusion 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

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.