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

Atherosclerosis
Steve E HumphriesSimon Broome Familial Hyperlipidaemia Register Group

Abstract

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. 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). 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 wi...Continue Reading

Citations

Nov 2, 2018·Current Opinion in Lipidology·Pedro MataLeopoldo Pérez de Isla
Sep 2, 2020·Current Atherosclerosis Reports·Jing PangGerald F Watts
Jun 23, 2019·Current Cardiology Reports·Ricardo SteinFernando Scolari
Apr 24, 2019·Current Cardiology Reports·Mahtab SharifiSteve E Humphries
Jan 25, 2020·Nature Reviews. Cardiology·Gerald F WattsKausik K Ray
Sep 3, 2020·Heart, Lung & Circulation·Jing PangUNKNOWN members of the FH Australasia Network Registry
Mar 4, 2021·Endocrinología, Diabetes Y Nutrición·Eva Nadiejda Gutiérrez-CortizoPedro Mata
Nov 30, 2018·Praxis·Manuela SchöbStefan Bilz
Mar 22, 2021·Nutrition, Metabolism, and Cardiovascular Diseases : NMCD·Elisenda ClimentJuan Pedro-Botet
Aug 7, 2021·Biomedicines·Juan Pedro-BotetDavid Benaiges
May 18, 2021·Expert Review of Endocrinology & Metabolism·Georgios PolychronopoulosKonstantinos Tziomalos
Nov 2, 2019·Progress in Cardiovascular Diseases·Marcio H Miname, Raul D Santos
Oct 8, 2021·Current Opinion in Lipidology·Dorothy F Graham, Frederick J Raal

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