Grant: Operating Grant

Background: Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease that is treated with repair in early childhood. Despite successful TOF repair in early childhood, risk of late mortality, related to pulmonary valve leaking, is cause for concern. Timing of pulmonary valve replacement surgery (PVR) is controversial and impact is not fully understood. Objective: The objective of our project is to study children and adults with repaired TOF across Canada to describe clinical and imaging characteristics of those receiving PVR, to evaluate practice patterns related to timing of PVR and to determine the impact of these variations on clinical outcome and quality of life. Study plan: We will create a registry of children and adults with repaired TOF followed in ten major Canadian congenital heart disease centres across the country: in Toronto, Montreal, Halifax, Edmonton and Vancouver. Through this Canadian-wide network we estimate that we will be able to follow close to 2000 patients with repaired TOF. We will review clinical measures, cardiac MRI studies, exercise tests and quality of life surveys. Through this cohort we will characterize who is referred for PVR surgery and for what indications, and in doing so, we aim to better understand the relationships between PVR surgery, cardiac magnetic imaging measurements, exercise testing, quality of life and overall survival in this population. Uniqueness of proposal: This study would be the first Canadian-wide collaborative study of children and adults born with congenital heart disease and would provide important information on how to improve the lives of thousands of Canadians with TOF and significant leaking of the pulmonary valve.