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

Autor: Humphries, Steve E., Cooper, Jackie A., Capps, Nigel, Durrington, Paul N., Jones, Ben, McDowell, Ian F.W., Soran, Handrean, Neil, Andrew H.W.
Rok vydání: 2018
Předmět:
Severe heterozygous familial
Adult
Male
Cardiac & Cardiovascular Systems
Coronary Disease
DIAGNOSIS
Risk Assessment
Severity of Illness Index
Article
Hyperlipoproteinemia Type II
Young Adult
Risk Factors
Humans
COHORT
Prospective Studies
Registries
1102 Cardiorespiratory Medicine and Haematology
Aged
RISK
Science & Technology
hypercholesterolemia
PLASMA
Severe heterozygous familial hypercholesterolemia
PCSK9 Inhibitors
Serine Endopeptidases
Heart
1103 Clinical Sciences
Cholesterol
LDL
Middle Aged
Prognosis
CANCER
United Kingdom
Coronary mortality
Peripheral Vascular Disease
DENSITY-LIPOPROTEIN CHOLESTEROL
Cardiovascular System & Hematology
Simon Broome Familial Hyperlipidaemia Register Group
Cardiovascular System & Cardiology
Female
Hydroxymethylglutaryl-CoA Reductase Inhibitors
Proprotein Convertase 9
Life Sciences & Biomedicine
Biomarkers
Zdroj: Atherosclerosis
ISSN: 1879-1484
Popis: 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/m2 (14.9% vs. 7.8%). The SMR for CHD mortality was significantly (p = 0.007) higher for SFH (220 (184–261) (34,134 person years, 129 deaths observed, vs. 59 expected) compared to NSFH of 144 (98–203) (15,432 person years, 32 observed vs. 22 expected). After adjustment for traditional risk factors, the Hazard Ratio for CHD mortality in SFH vs. NSFH was 1.22 (0.80–1.87) p = 0.36, indicating that the excess risk was largely accounted for by these factors. Conclusions CHD mortality remains elevated in treated FH, especially for SFH, emphasising the importance of optimal lipid-lowering and management of other risk factors.
Graphical abstract Image 1
Highlights • Patients with IAS-defined “severe” FH (SFH) are at highest risk of future CHD. • In the UK Simon Broome FH Register ∼70% meet the SFH criteria. • Those with SFH have 64% higher CHD mortality than non SFH patients. • This is explained by their higher classical risk factors including untreated LDL-C.
Databáze: OpenAIRE
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