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). The HiRO Hypertrophic Cardiomyopathy registry, biobank and imaging data repository (HiRO-HCM) is a multicenter study that will prospectively enroll patients with HCM as well as those carrying sarcomeric gene variants predisposing to HCM.
The objectives of HiRO-HCM are:
1. to better understand the natural history of the disease and identify clinical markers and biomarkers for adverse outcomes; 2. to derive and validate risk prediction models for disease expression, complications and response to therapy; 3. to better define the genetic architecture of sarcomeric and non-sarcomeric HCM.
Clinical diagnosis of HCM, defined as
maximal LVWT ≥15mm, or
maximal LVWT ≥13mm, in presence of a diagnosis of first degree relative with HCM, or
septal wall thickness with z-score >2 in a child
Clinical or molecular diagnosis of Noonan syndrome or other Rasopathies
Clinical or molecular diagnosis of metabolic disease associated with cardiomyopathy, such as Pompe (GAA), Fabry (GLA), Danon (LAMP2), AMP-kinase (PRKAG2), and carnitine disorders
Clinical diagnosis of a neuromuscular disease associated with cardiomyopathy, such as Friedrich's ataxia
Clinical diagnosis of cardiac amyloidosis with or without the presence of genetic variants in TTR
The HiRO-HCM registry, biobank and imaging data repository is a multicenter collaboration that will prospectively enroll patients with HCM as well as those carrying genetic variants predisposing to HCM.
Family-based
Prospective
Not listed
Create a Canadian Research Database, a biobank and an imaging data repository for those affected by hypertrophic cardiomyopathy (HCM) or carrying a sarcomeric gene variant associated with HCM
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Montreal Heart Institute
Lead sponsor
Canadian Institutes of Health Research (CIHR)
Collaborator
