Homozygous Familial Hypercholesterolemia in Canada: An Observational Study
Study context
- Population
- Patients with homozygous familial hypercholesterolemia (HoFH) in Canada, Familial Hypercholesterolemia Canada network academic sites
- Participants
- 48 patients
- Outcome
- Clinical characteristics and CV outcomes
Methodological detailsmulticentre, national registry, Clinical and genetic data collected via standardized questionnaire sent to academic sites
- Multicentre
- National registry
- Clinical and genetic data collected via standardized questionnaire sent to academic sites
Main finding
As reported in the abstract
- Median age at diagnosis (IQR 5-24)
- 12 years
- Untreated LDL-C, median (IQR 10.5-18.6 mmol/L; 580 mg/dL, IQR 404-717 mg/dL)
- 15.0 mmol/L
- Median age at last follow-up (IQR 26-54 years)
- 40 years
- Treated LDL-C (IQR 4.73-9.51 mmol/L; 261 mg/dL, IQR 183-368 mg/dL)
- 6.75 mmol/L
- On statins
- 95.5%
- On ezetimibe
- 88.6%
- On PCSK9 inhibitors
- 34.1%
- On lomitapide
- 27.3%
- On evinacumab
- 13.6%
- LDL apheresis or plasmapheresis
- 56.8%
- Deaths
- 7 (14.5%)
- MACE
- 14.6%
- Average age at MACE onset (IQR 20-36 years)
- 30 years
- Aortic stenosis
- 47.9%
- Aortic valve replacement
- 10 (20.8%)
Observational data: associations, not effects.
Original publication
Homozygous Familial Hypercholesterolemia in Canada: An Observational Study. JACC Adv. Published 26 April 2023. doi:10.
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