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Coronary heart disease mortality in treated familial hypercholesterolaemia: Update of the UK Simon Broome FH register

Humphries, SE; Cooper, JA; Seed, M; Capps, N; Durrington, PN; Jones, B; McDowell, IFW; ... Neil, HAW; + view all (2018) Coronary heart disease mortality in treated familial hypercholesterolaemia: Update of the UK Simon Broome FH register. Atherosclerosis , 274 pp. 41-46. 10.1016/j.atherosclerosis.2018.04.040. Green open access

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Abstract

BACKGROUND AND AIMS: Patients with familial hypercholesterolaemia (FH) have an elevated risk of coronary heart disease (CHD). Here we compare changes in CHD mortality in patients with heterozygous (FH) pre 1992, before lipid-lowering therapy with statins was used routinely, and in the periods 1992–2008 and 2008–2016. METHODS: 1903 Definite (DFH) and 1650 Possible (PFH) patients (51% women) aged 20–79 years, recruited from 21 lipid clinics in the United Kingdom and followed prospectively between 1980 and 2016 for 67,060 person-years. The CHD standardised mortality ratio (SMR) compared to the population in England and Wales was calculated (with 95% Confidence intervals). RESULTS: There were 585 deaths, including 252 from CHD. Overall, the observed 2.4-fold excess coronary mortality for treated DFH post-1991 was significantly higher than the 1.78 excess for PFH (35% 95% CI 3%–76%). In patients with DFH and established coronary disease, there was a significant excess coronary mortality in all time periods, but in men it was reduced from a 4.83-fold excess (2.32–8.89) pre-1992 to 4.66 (3.46–6.14) in 1992–2008 and 2.51 (1.01–5.17) post-2008, while in women the corresponding values were 7.23 (2.65–15.73), 4.42 (2.70–6.82) and 6.34 (2.06–14.81). Primary prevention in men with DFH resulted in a progressive reduction in coronary mortality over the three time-periods, with no excess mortality evident post-2008 (0.89 (0.29–2.08)), although in women the excess persisted (post-2008 3.65 (1.75–6.72)). CONCLUSIONS: The results confirm the benefit of statin treatment in reducing CHD mortality, but suggest that FH patients with pre-existing CHD and women with FH may not be treated adequately.

Type: Article
Title: Coronary heart disease mortality in treated familial hypercholesterolaemia: Update of the UK Simon Broome FH register
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.atherosclerosis.2018.04.040
Publisher version: https://doi.org/10.1016/j.atherosclerosis.2018.04....
Language: English
Additional information: © 2018 The Authors. Published by Elsevier B.V. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/)
Keywords: Heterozygous familial hypercholesterolemia, Coronary mortality, Cancer mortality, Dutch lipid clinic network score
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
URI: https://discovery.ucl.ac.uk/id/eprint/10054723
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