Objective
To create a Canadian research database and biobank designed to collect healthcare information and samples on patients being investigated and treated for inherited heart rhythm conditions. This provides a means to study inherited heart rhythm conditions and build an accurate picture of this population in Canada, engaging more than 90% of inherited heart rhythm patients in clinical and translational research.
Background
The Hearts in Rhythm Organization (HiRO) is a national network of Canadian researchers and 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-samples in a national data registry and biobank, hoping to improve the detection and treatment of inherited heart rhythm disorders and prevent sudden death.
Study Outcomes
Primary
- Engage 90+% of inherited heart rhythm patients in clinical / translational research
- Develop a 10,000-case registry of consenting participants at genetic risk for sudden death and their first-degree relatives
Secondary
- Develop new care pathways including case finding, risk-assessment algorithms, and improved prevention and treatment guidance
- Develop a patient education curriculum and educational materials
- Develop a system/tool to measure clinical outcomes
- Create a national research database for inherited heart rhythm conditions
- Improve the efficiency and accessibility of research participation for all willing Canadians, including bio-sample storage
- Improve care for inherited heart rhythm patients
- Provide optional biobanking of DNA and serum for future research
Eligibility Criteria
The criteria below are a summary. Your study doctor will confirm whether this study is right for you.
Inclusion Criteria
- Recognized genetic syndromes: Long QT (LQT), Short QT (SQT), CPVT, Brugada (BrS), ARVC, Familial Cardiac Conduction Disease (FCCD)
- Deceased cases of SCD suspicious for an inherited heart rhythm condition, with signed consent from next of kin
- Inherited heart rhythm patients referred for risk of SCD (including first- and second-degree relatives)
- SCD syndromes seen in clinic: unexplained cardiac arrest, early repolarization, idiopathic VF, short-coupled IVF, PMVT NYD, and SADS
- Mendelian cardiomyopathies: HCM, Mendelian dilated cardiomyopathy (incl. LMNA & PLN), and LVNC
- Carriers of a pathogenic or likely-pathogenic variant for an inherited arrhythmia or cardiomyopathy gene
Exclusion Criteria
- Unwilling or 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 without a positive family history
- Aortopathies including Marfan syndrome, Ehlers-Danlos, and familial thoracic aortic aneurysm/dissection
- Neuromuscular disease
- Familial hypercholesterolemia
About taking part
If you are considering joining this study, please review the Letter of Information below. It explains the study in detail, including what participation involves, the potential risks and benefits, and your rights as a participant.
HiRO Registry Letter of Information