Cardiovascular disease incidence in 21 years follow-up in severe and non-severe familial hypercholesterolaemia (FH) : Data from the UK Simon Broome FH register (2020)

Type of publication:
Conference abstract

Author(s):
Iyen B.; Qureshi N.; Weng S.; Roderick P.; *Capps N.; Durrington P.; Mcdowell I.; Soran H.; Neil A.; Humphries S.E.

Citation:
Atherosclerosis; Dec 2020; vol. 315

Abstract:
Background and Aims: The Simon Broome (SB) FH register has previously reported a 2.2-fold higher Cardiovascular Disease (CVD) mortality in those with “severe-FH” (SFH) compared to “non-severe-FH” (NSFH). Here we examine CVD morbidity over 21 years follow-up, by linking the register participants with the UK secondary care Hospital Episode Statistics (HES) database.
Method(s): SFH and NSFH were as defined by the 2016 International Atherosclerosis Society criteria. Patients aged 20-79  years (52% female) were recruited from 21 UK lipid clinics and followed between 1997-2018. Outcomes analysed were composite CVD (first HES outcome of coronary heart disease (CHD), myocardial infarction, stable or unstable angina, stroke, TIA, PVD, heart failure, PCI and CABG) and then CVD subtypes. The excess Standardised Morbidity Ratio (SMbR) compared to an age-matched UK general practice sample was calculated (95% Confidence intervals).
Result(s): Of the 3553 SB register subjects, linkage with HES was available for 2988 (84%) participants, of whom 1,646 (66.7%) met the SFH definition. Overall the composite CVD SMbR was 6.55(6.20-6.92). In the SFH group (27,680 pyrs follow-up and 762 events) the SMbR for any CVD event was 9.38 (8.74-10.07), while in the NSFH group (13,750 pyrs follow-up and 237 events) was 5.87(5.17-6.67). For CHD the estimates were 11.88(11.01-12.82) vs 7.38(6.45-8.47) respectively.
Conclusion(s): CVD morbidity in conventionally treated FH patients was over 6-fold higher than the general population, with rates in those with SFH 60% higher than those with NSFH. This emphasises the potential value of more intensive lipid-lowering, and management of other risk factors for those with FH.

Cardiovascular disease incidence in 21 years follow-up in severe and non-severe familial hypercholesterolaemia (FH) : Data from the UK Simon Broome FH register (2020)

Type of publication:
Conference abstract

Author(s):
Iyen B.; Qureshi N.; Weng S.; Roderick P.; *Capps N.; Durrington P.; Mcdowell I.; Soran H.; Neil A.; Humphries S.E.

Citation:
Atherosclerosis; December 2020; vol. 315

Abstract:
Background: The Simon Broome (SB) FH register has previously reported a 2.2-fold higher Cardiovascular Disease (CVD) mortality in those with “severe-FH” (SFH) compared to “non-severe-FH” (NSFH). Here we examine CVD morbidity over 21 years follow-up, by linking the register participants with the UK secondary care Hospital Episode Statistics (HES) database.
Method(s): SFH and NSFH were as defined by the 2016 International Atherosclerosis Society criteria. Patients aged 20-79 years (52% female) were recruited from 21 UK lipid clinics and followed between 1997-2018. Outcomes analysed were composite CVD (first HES outcome of coronary heart disease (CHD), myocardial infarction, stable or unstable angina, stroke, TIA, PVD, heart failure, PCI and CABG) and then CVD subtypes. The excess Standardised Morbidity Ratio (SMbR) compared to an age-matched UK general practice sample was calculated (95% Confidence intervals).
Result(s): Of the 3553 SB register subjects, linkage with HES was available for 2988 (84%) participants, of whom 1,646 (66.7%) met the SFH definition. Overall the composite CVD SMbR was 6.55(6.20-6.92). In the SFH group (27,680 pyrs follow-up and 762 events) the SMbR for any CVD event was 9.38 (8.74-10.07), while in the NSFH group (13,750 pyrs follow-up and 237 events) was 5.87(5.17-6.67). For CHD the estimates were 11.88(11.01-12.82) vs 7.38(6.45-8.47) respectively.
Conclusion(s): CVD morbidity in conventionally treated FH patients was over 6-fold higher than the general population, with rates in those with SFH 60% higher than those with NSFH. This emphasises the potential value of more intensive lipid-lowering, and management of other risk factors for those with FH.

Sex differences in cardiovascular morbidity associated with familial hypercholesterolaemia: A retrospective cohort study of the UK Simon Broome register linked to national hospital records (2020)

Type of publication:
Journal article

Author(s):
Barbara Iyen, Nadeem Qureshi, Stephen Weng, Paul Roderick, Joe Kai, *Nigel Capps, Paul N. Durrington, Ian FW. McDowell, Handrean Soran, Andrew Neil, Steve E. Humphries

Citation:
Atherosclerosis, Nov 2020 [epub ahead of print]

Abstract:
Background and aims: The UK Simon Broome (SB) familial hypercholesterolaemia (FH) register previously reported 3-fold higher standardised mortality ratio for cardiovascular disease (CVD) in women compared to men from 2009 to 2015. Here we examined sex differences in CVD morbidity in FH by national linkage of the SB register with Hospital Episode Statistics (HES).
Methods: Of 3553 FH individuals in the SB register (aged 20–79 years at registration), 2988 (52.5% women) had linked HES records. Standardised Morbidity Ratios (SMbR) compared to an age and sex-matched UK general practice population were calculated [95% confidence intervals] for first CVD hospitalisation in HES (a composite of coronary heart disease (CHD), myocardial infarction (MI), stable or unstable angina, stroke, TIA, peripheral vascular disease (PVD), heart failure, coronary revascularisation interventions).
Results: At registration, men had significantly (p < 0.001) higher prevalence of previous CHD (24.8% vs 17.6%), previous MI (13.2% vs 6.3%), and were commenced on lipid-lowering treatment at a younger age than women (37.5 years vs 42.3 years). The SMbR for composite CVD was 6.83 (6.33–7.37) in men and 7.55 (6.99–8.15) in women. In individuals aged 30–50 years, SMbR in women was 50% higher than in men (15.04 [12.98–17.42] vs 10.03 [9.01–11.17]). In individuals >50 years, SMbR was 33% higher in women than men (6.11 [5.57–6.70] vs 4.59 [4.08–5.15]).
Conclusions: Excess CVD morbidity due to FH remains markedly elevated in women at all ages, but especially those aged 30–50 years. This highlights the need for earlier diagnosis and optimisation of lipid-lowering risk factor management for all FH patients, with particular attention to young women with FH.

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HEART UK consensus statement on Lipoprotein(a): A call to action (2019)

Type of publication:
Journal article

Author(s):
Cegla J.; Neely R.D.G.; France M.; Ferns G.; Byrne C.D.; Halcox J.; Datta D.; *Capps N.; Shoulders C.; Qureshi N.; Rees A.; Main L.; Payne J.; Cramb R.; Viljoen A.; Soran H.

Citation:
Atherosclerosis; Dec 2019; vol. 291 ; p. 62-70

Abstract:
Lipoprotein(a), Lp(a), is a modified atherogenic low-density lipoprotein particle that contains apolipoprotein(a). Its levels are highly heritable and variable in the population. This consensus statement by HEART UK is based on the evidence that Lp(a) is an independent cardiovascular disease (CVD) risk factor, provides recommendations for its measurement in clinical practice and reviews current and emerging therapeutic strategies to reduce CVD risk. Ten statements summarise the most salient points for practitioners and patients with high Lp(a). HEART UK recommends that Lp(a) is measured in adults as follows: 1) those with a personal or family history of premature atherosclerotic CVD; 2) those with first-degree relatives who have Lp(a) levels >200 nmol/l; 3) patients with familial hypercholesterolemia; 4) patients with calcific aortic valve stenosis and 5) those with borderline (but <15%) 10-year risk of a cardiovascular event. The management of patients with raised Lp(a) levels should include: 1) reducing overall atherosclerotic risk; 2) controlling dyslipidemia with a desirable non-HDL-cholesterol level of <100 mg/dl (2.5 mmol/l) and 3) consideration of lipoprotein apheresis.

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Corrigendum: It has been brought to our attention that the wording of the German reimbursement criteria for apheresis is not clear in the above paper (Section 7. Management of patients with raised Lipoprotein(a), Subheading: Apheresis). This should read: “In Germany, Lp(a) levels exceeding 60 mg/dl and LDL-cholesterol in normal range along with progressive CVD has been approved as an indication for regular lipoprotein apheresis since 2008.”

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