Project 462651

Sudden death is a devastating outcome of hypertrophic cardiomyopathy, a genetic disease of heart muscle. It is the #1 cause of sudden death in young athletes. Sudden death can be prevented by timely insertion of a life-saving device called an implantable cardioverter-defibrillator (ICD). However, until recently, we lacked tools to accurately predict who is high-risk and should get this device. An international team led by us developed the first digital tool that provides a personalized risk for sudden death based on a combination of clinical and genetic factors unique to each patient. We will launch this tool into hospital electronic health records, where it will pull information and generate risk scores for each patient. This requires developing a strategy for implementation that ensures that the tool is user-friendly and is consistently used in eligible patients. Each year across Canada, close to a hundred children and youth with hypertrophic cardiomyopathy will suffer a sudden death or near-death event. Our current practice only protects 20% of these young lives. Effective implementation of the tool will help protect more young lives through timely ICDs so that families avoid having to suffer the devastating loss of a young child.