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European Heart Journal Advance Access originally published online on October 7, 2008
European Heart Journal 2008 29(21):2625-2633; doi:10.1093/eurheartj/ehn422
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org
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Reductions in all-cause, cancer, and coronary mortality in statin-treated patients with heterozygous familial hypercholesterolaemia: a prospective registry study

Andrew Neil1,*, Jackie Cooper2, John Betteridge3, Nigel Capps4, Ian McDowell5, Paul Durrington6, Mary Seed7, Steve E. Humphries2 on behalf of the Simon Broome Familial Hyperlipidaemia Register Group

1 NIHR School of Primary Care Research, Division Public Health and Primary Health Care, University of Oxford, Old Road Headington, Oxford OX3 7LF, UK
2 Centre for Cardiovascular Genetics, British Heart Foundation Laboratories, Royal Free and University College London Medical School, 5 University Street, London WC1E 6JJ, UK
3 Department of Medicine, Royal Free and University College London Medical School, London, UK
4 Department of Clinical Biochemistry, The Shrewsbury and Telford Hospital NHS Trust, Princess Royal Hospital, Telford, UK
5 Department of Medical Biochemistry and Immunology, University Hospital of Wales, Cardiff, UK
6 Cardiovascular Research Group, School of Clinical and Laboratory Sciences, University of Manchester, Manchester, UK
7 Department of Medicine, Imperial College Faculty of Medicine, Charing Cross Hospital, University of London, London, UK

Received 11 July 2008; revised 25 August 2008; accepted 29 August 2008; online publish-ahead-of-print 7 October 2008.

* Corresponding author. Tel +44 1865 289 258, Fax: +44 1865 289 258. Email: andrew.neil{at}wolfson.ox.ac.uk

See page 2583 for the editorial comment on this article (doi:10.1093/eurheartj/ehn448)

Aims: To examine the changes in coronary, all-cause, and cancer mortality in patients with heterozygous familial hypercholesterolaemia (FH) before and after lipid-lowering therapy with statins.

Methods and results: A total of 3382 patients (1650 men) aged <80 years were recruited from 21 lipid clinics in the United Kingdom and followed prospectively between 1980 and 2006 for 46 580 person-years. There were 370 deaths, including 190 from coronary heart disease (CHD) and 90 from cancer. The standardized mortality ratio (compared with the population in England and Wales) was calculated before and from 1 January 1992. In patients aged 20–79 years, CHD mortality fell significantly by 37% (95% CI = 7–56) from 3.4- to 2.1-fold excess. Primary prevention resulted in a 48% reduction in CHD mortality from 2.0-fold excess to none, with a smaller reduction of nearly 25% in patients with established disease. Coronary mortality was reduced more in women than in men. In patients without known CHD at registration, all-cause mortality from 1992 was 33% (21–43), lower than in the general population, mainly due to a 37% (21–50) lower risk of fatal cancer.

Conclusion: The results emphasize the importance of early identification of FH and treatment with statins.

Key Words: Familial hypercholesterolaemia • Coronary heart disease • All-cause mortality • Cancer mortality


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