Coronary heart disease mortality in severe vs. non-severe familial hypercholesterolaemia in the Simon Broome Register.


Journal

Atherosclerosis
ISSN: 1879-1484
Titre abrégé: Atherosclerosis
Pays: Ireland
ID NLM: 0242543

Informations de publication

Date de publication:
02 2019
Historique:
received: 24 08 2018
revised: 15 10 2018
accepted: 08 11 2018
pubmed: 22 11 2018
medline: 14 4 2020
entrez: 22 11 2018
Statut: ppublish

Résumé

The International Atherosclerosis Society (IAS) has proposed that patients with "severe" FH (SFH) would warrant early and more aggressive cholesterol-lowering treatment such as with PCSK9 inhibitors. SFH is diagnosed if LDL-cholesterol (LDLC) > 10 mmol/L, or LDLC >8.0 mmol/L plus one high-risk feature, or LDLC >5 mmol/L plus two high-risk features. Here we compare CHD mortality in SFH and non-SFH (NSFH) patients in the UK prospective Simon Broome Register since 1991, when statin use became routine. 2929 definite or possible PFH patients (51% women) aged 20-79 years were recruited from 21 UK lipid clinics and followed prospectively between 1992 and 2016. The excess CHD standardised mortality ratio (SMR) compared to the England and Wales population was calculated (with 95% confidence intervals). 1982 (67.7%) patients met the SFH definition. Compared to the non-SFH, significantly (p < 0.001) more SFH patients had diagnosed CHD at baseline (24.6% vs. 17.5%), were current smokers (21.9% vs 10.2%) and had a BMI > 30 kg/m CHD mortality remains elevated in treated FH, especially for SFH, emphasising the importance of optimal lipid-lowering and management of other risk factors.

Sections du résumé

BACKGROUND AND AIMS
The International Atherosclerosis Society (IAS) has proposed that patients with "severe" FH (SFH) would warrant early and more aggressive cholesterol-lowering treatment such as with PCSK9 inhibitors. SFH is diagnosed if LDL-cholesterol (LDLC) > 10 mmol/L, or LDLC >8.0 mmol/L plus one high-risk feature, or LDLC >5 mmol/L plus two high-risk features. Here we compare CHD mortality in SFH and non-SFH (NSFH) patients in the UK prospective Simon Broome Register since 1991, when statin use became routine.
METHODS
2929 definite or possible PFH patients (51% women) aged 20-79 years were recruited from 21 UK lipid clinics and followed prospectively between 1992 and 2016. The excess CHD standardised mortality ratio (SMR) compared to the England and Wales population was calculated (with 95% confidence intervals).
RESULTS
1982 (67.7%) patients met the SFH definition. Compared to the non-SFH, significantly (p < 0.001) more SFH patients had diagnosed CHD at baseline (24.6% vs. 17.5%), were current smokers (21.9% vs 10.2%) and had a BMI > 30 kg/m
CONCLUSIONS
CHD mortality remains elevated in treated FH, especially for SFH, emphasising the importance of optimal lipid-lowering and management of other risk factors.

Identifiants

pubmed: 30458964
pii: S0021-9150(18)31469-2
doi: 10.1016/j.atherosclerosis.2018.11.014
pmc: PMC6403443
pii:
doi:

Substances chimiques

Biomarkers 0
Cholesterol, LDL 0
Hydroxymethylglutaryl-CoA Reductase Inhibitors 0
PCSK9 Inhibitors 0
PCSK9 protein, human EC 3.4.21.-
Proprotein Convertase 9 EC 3.4.21.-
Serine Endopeptidases EC 3.4.21.-

Types de publication

Comparative Study Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

207-212

Subventions

Organisme : Medical Research Council
ID : MR/K023667/1
Pays : United Kingdom
Organisme : British Heart Foundation
ID : RG3008
Pays : United Kingdom
Organisme : British Heart Foundation
ID : RG008/08
Pays : United Kingdom

Commentaires et corrections

Type : CommentIn

Informations de copyright

Copyright © 2018 The Authors. Published by Elsevier B.V. All rights reserved.

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Auteurs

Steve E Humphries (SE)

Centre for Cardiovascular Genetics, Institute of Cardiovascular Science, University College London, University Street, London, WC1E 6JJ, UK. Electronic address: steve.humphries@ucl.ac.uk.

Jackie A Cooper (JA)

Centre for Cardiovascular Genetics, Institute of Cardiovascular Science, University College London, University Street, London, WC1E 6JJ, UK.

Nigel Capps (N)

Department of Clinical Biochemistry, The Shrewsbury and Telford Hospital NHS Trust, Princess Royal Hospital, Telford, UK.

Paul N Durrington (PN)

Cardiovascular Research Group, School of Clinical and Laboratory Sciences, University of Manchester, Manchester, UK.

Ben Jones (B)

Section of Investigative Medicine, Imperial College London, London, UK.

Ian F W McDowell (IFW)

Department of Medical Biochemistry and Immunology, University Hospital of Wales, Cardiff, UK.

Handrean Soran (H)

University Department of Medicine, Manchester University Hospitals NHS Foundation Trust, Manchester, UK.

Andrew H W Neil (AHW)

Wolfson College, University of Oxford, Oxford, UK.

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Classifications MeSH