title: Contemporary Trends in the Management and Outcomes of Patients With Familial Hypercholesterolemia in Canada: A Prospective Observational Study.
abstract: Heterozygous familial hypercholesterolemia (HeFH) is one of the most common genetic diseases in the world and an important cause of premature cardiovascular (CV) disease. The purpose of this study was to characterize the clinical features, current treatment patterns, and CV outcomes of patients with HeFH in British Columbia, Canada.
  
  
  
  We conducted a longitudinal observational study of patients with HeFH attending a specialized lipid clinic. We collected data on lipid levels, medication use, and CV events at baseline and last follow-up.
  
  
  
  We recruited 339 patients with clinically diagnosed HeFH, with a total of 3700 person-years of follow-up. The mean low-density lipoprotein cholesterol (LDL-C) level was 5.9 mmol/L at baseline and 3.7 mmol/L at last follow-up. Use of lipid-lowering therapy (LLT) increased from 35.7% at baseline to 84.7% at last follow-up. A ≥ 50% reduction in LDL-C level was achieved in 34.5% of patients, and an LDL-C level ≤ 2 mmol/L was seen in 8.3%. The overall CV event rate in this cohort was 33.5/1000 person-years. Among patients who had a CV event during follow-up, 59% experienced a recurrent event within 5 years.
  
  
  
  These data contribute to our understanding of contemporary trends in the management of patients with HeFH in Canada. Despite a majority of patients receiving LLT, few patients reached high-risk lipid targets. These data highlight important opportunities to improve the care of patients with HeFH.
authors.affiliation: Healthy Heart Program Prevention Clinic, St. Paul's Hospital, Vancouver, British Columbia, Canada; Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada; Centre for Heart Lung Innovation, Providence Health Care Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Electronic address: liam.brunham@ubc.ca.
authors.forename: Liam R
authors.initials: LR
authors.lastname: Brunham
authors.affiliation: Healthy Heart Program Prevention Clinic, St. Paul's Hospital, Vancouver, British Columbia, Canada; Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, British Columbia, Canada.
authors.forename: Lubomira
authors.initials: L
authors.lastname: Cermakova
authors.affiliation: Centre for Health Evaluation and Outcome Sciences, Providence Health Care Research Institute, University of British Columbia, Vancouver, British Columbia, Canada.
authors.forename: Terry
authors.initials: T
authors.lastname: Lee
authors.affiliation: Healthy Heart Program Prevention Clinic, St. Paul's Hospital, Vancouver, British Columbia, Canada.
authors.forename: Ida
authors.initials: I
authors.lastname: Priecelova
authors.affiliation: Sanofi-Aventis Canada, Inc, Laval, Quebec, Canada.
authors.forename: Karine
authors.initials: K
authors.lastname: Alloul
authors.affiliation: Sanofi-Aventis Canada, Inc, Laval, Quebec, Canada.
authors.forename: Marilyn
authors.initials: M
authors.lastname: de Chantal
authors.affiliation: Healthy Heart Program Prevention Clinic, St. Paul's Hospital, Vancouver, British Columbia, Canada; Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada; Centre for Heart Lung Innovation, Providence Health Care Research Institute, University of British Columbia, Vancouver, British Columbia, Canada.
authors.forename: Gordon A
authors.initials: GA
authors.lastname: Francis
authors.affiliation: Healthy Heart Program Prevention Clinic, St. Paul's Hospital, Vancouver, British Columbia, Canada; Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, British Columbia, Canada.
authors.forename: Jiri
authors.initials: J
authors.lastname: Frohlich
mesh_terms: D000328:Adult; D000924:Anticholesteremic Agents / Q000627:therapeutic use*; D001955:British Columbia / Q000453:epidemiology; D008078:Cholesterol, LDL / Q000097:blood*; D019468:Disease Management; D005260:Female; D005500:Follow-Up Studies; D005544:Forecasting; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood / Q000188:drug therapy* / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies
chemical_list: D000924:Anticholesteremic Agents; D008078:Cholesterol, LDL