biostudies-literatureHumphries SEUCLH NIHR BRCAstra Zeneca and Schering-Plough LtdBritish Heart FoundationMedical Research CouncilNational Institute for Health Research (NIHR)Pfizer LtdAcademy of Medical SciencesNIHR Clinical Lectureship41-46https://www.ebi.ac.uk/biostudies/studies/S-EPMC6013645biostudies-literatureUnknown274<h4>Background and aims</h4>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.<h4>Methods</h4>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).<h4>Results</h4>There were 585 deaths, including 252 from CHD. Overall, the observed 2.4-fold excess coronary AtherosclerosisCoronary heart disease mortality in treated familial hypercholesterolaemia: Update of the UK Simon Broome FH register.PMC6013645CL-2017-21-002RG008/08SGL019\1078RG3008MR/K023667/1ACF-2010-21-012Durrington PNCapps NMcDowell IFWHumphries SENeil HAWSoran HSimon Broome Familial Hyperlipidaemia Register GroupCooper JAJones BSeed MfalseCoronary heart disease mortality in treated familial hypercholesterolaemia: Update of the UK Simon Broome FH register.<h4>Background and aims</h4>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.<h4>Methods</h4>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).<h4>Results</h4>There were 585 deaths, including 252 from CHD. Overall, the observed 2.4-fold excess coronary 2018-01-01T00:00:00Z2018 Jul2025-05-29T21:20:45.514Z2025-05-29T21:20:45.514ZS-EPMC60136452975128310.1016/j.atherosclerosis.2018.04.040