Coronary heart disease mortality in severe vs. non-severe familial hypercholesterolaemia in the Simon Broome Register (2019)

Type of publication:
Journal article

Author(s):
Humphries, Steve E; Cooper, Jackie A; *Capps, Nigel; Durrington, Paul N; Jones, Ben; McDowell, Ian F W; Soran, Handrean; Neil, Andrew H W; Simon Broome Familial Hyperlipidaemia Register Group

Citation:
Atherosclerosis. 2019 Feb;281:207-212

Abstract:
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)>10mmol/L, or LDLC >8.0mmol/L plus one highrisk feature, or LDLC >5mmol/L plus two high-risk features. Here we compare CHD mortality in SFH and nonSFH (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).RESULTS1982 (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>30kg/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.

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Patient experience within the adult congenital heart disease outreach network: A questionnaire-based study (2018)

Type of publication:
Journal article

Author(s):

Georgina Ooues, Paul Clift, Sarah Bowater, Sayqa Arif, Andrew Epstein, Neeraj Prasad, Dawn Adamson, Mandy Cummings, Charles Spencer, Paul Woodmansey, Jenny Borley, *Thomas Ingram, Adrian Morley-Davies, William Roberts, Najmi Qureshi, Susan Hawkesford, Nichola Pope, James Anthony, Thomas Gaffey, Sara Thorne, Lucy Hudsmith and On behalf of The West Midlands ACHD Network, UK

Citation:
Journal of Congenital Cardiology; Sep 2018; vol. 2 (no. 1)

Abstract:
Background: Specialist multi-disciplinary care improves outcomes of Adult Congenital Heart Disease (ACHD) patients. Following the NHS England Congenital Heart Disease standards review, the aim is to deliver high quality, patient-centred, care closer to patients’ homes. Cardiac investigations performed on the same day of outpatient appointments reduce the non-attendance rates. This young cohort of patients, benefits from comprehensive multi-disciplinary management. We developed a Patient Questionnaire across our West Midlands ACHD network to measure patient experience. Methods: Patient questionnaires were distributed to patients attending outpatient clinics in all 8 Outreach Centres and the Level 1 ACHD Centre (University Hospitals Birmingham). Results: 71 males (55%) and 59 females (45%), median age range 25-34years old (range between 16 and 75years old), returned the questionnaires (n=130). Most patients travelled less than one hour to hospital (93%, n=120) and less than 20miles (86%, n=99). The mean travel distance was 14+/-12.3miles (range 1 to 160miles), with Level 1 ACHD Centre patients travelling a significantly longer distance (mean 29.6+/-44miles) compared to the local Outreach Centres (mean 11.3+/-9miles, p=0.0037). There was a wide variability in the provision of parking, although most patients found the appointment time and location convenient (91%, n=117 and 95%, n=121 respectively). There was also marked variation in the number of electrocardiograms (19-100%) and echocardiograms (0-60%) performed on the same day as their clinic appointment. Most patients felt they were given enough information regarding their condition (85%, n=98), with no significant differences between the centres (p=0.24). Conclusion: To our knowledge, this is the first questionnaire-based study assessing patient experience within the NHS ACHD Outreach network with significantly reduced travel times and maintained high patient satisfaction. There was a wide variation ininvestigations performed and patient information leaflets provided. Standardisation of services is required at allcentres to ensure equity of care, with Specialist Nurses’ input and more availability of tests on the day of clinicappointments in all centres.

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Coronary heart disease mortality in treated familial hypercholesterolaemia: Update of the UK Simon Broome FH register (2018)

Type of publication:
Journal article

Author(s):
Humphries S.E.; Cooper J.A.; Seed M.; *Capps N.; Durrington P.N.; Jones B.; McDowell I.F.W.; Soran H.; Neil H.A.W.

Citation:
Atherosclerosis; Jul 2018; vol. 274 ; p. 41-46

Abstract:
Background and aims: 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. Methods: 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). Results: 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 with DFH resulted in a progressive reduction in coronary mortality over the three time-periods, with no excess mortality evident post-2008 (0.89 (0.29-2.08)), although in women the excess persisted (post-2008 3.65 (1.75-6.72)). Conclusions: The results confirm the benefit of statin treatment in reducing CHD mortality, but suggest that FH patients with pre-existing CHD and women with FH may not be treated adequately.

Optimising patient experience within the ACHD outreach network: A questionnaire based study (2018)

Type of publication:
Conference abstract

Author(s):
Ooues G.; Clift P.; Bowater S.; Arif S.; Hawkesford S.; Pope N.; Anthony J.; Gaffey T.; Thorne S.; Hudsmith L.; Epstein A.; Prasad N.; Adamson D.; Cummings M.; Spencer C.; Woodmansey P.; *Ingram T.; Morley-Davies A.; Roberts W.; Qureshi N.

Citation:
Heart; Feb 2018; vol. 104, Suppl 2, A12

Abstract:
Purpose The NHS England Congenital Heart Disease standards review is based on a network model to deliver high quality, safe and effective services as locally as possible. We developed a Patient Questionnaire across our Adult Congenital Heart Disease (ACHD) West Midlands network to measure patient experience, satisfaction and to improve services across the network. Methods Patient questionnaires were distributed to patients in all 8 Outreach and the Level 1 ACHD Centre (University Hospital Birmingham). Data was analysed including patients’ replies on travel to outpatient clinic, satisfaction on location and timing of their appointment, review by ACHD Specialist Nurse and tests performed, information on their condition and leaflets provided and patients’ demographics. Results 130 questionnaires were returned. The majority of patients (67%, n=87) travelled to their appointment with their own car, either alone (36%, n=46) or with a member of their family (44%, n=56). Most patients (93%, n=120) travelled less than one hour to hospital and less than 20 miles (86%, n=99). Patients attending Level 1 Centre appointments travelled a longer distance (mean 29.6+/-44 miles) compared to the Outreach Centres (mean 9.9+/-2.8 miles). Almost all patients found the appointment time and location convenient for them (91%, n=117% and 95%, n=121), and were given enough information regarding their condition (85%, n=98). Conclusion With the development of ACHD Network Outreach clinics to facilitate services and appointments closer to patients’ homes, travel times are reduced and high patient satisfaction is maintained.

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Coronary heart disease mortality in treated Familial Hypercholesterolaemia: Update of the UK Simon Broome FH Register (2017)

Type of publication:
Conference abstract

Author(s):
Humphries S.E.; Cooper J.A.; Seed M.; *Capps N.; Durrington P.N.; Jones B.; McDowell I.F.W.; Soran H.; Neil

Citation:
Atherosclerosis Supplements; 2017; vol. 28, Pages 41-46

Abstract:
Background: Guidelines for the management of patients with Familial Hypercholesterolaemia (FH) recommend the use of high intensity statin therapy to reduce subsequent 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 till the present. Methods: Analysis used 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 1980-1991 (6627 personyears) 1992-2008 (43117 person-years) and 2009-2016 (17317 person-years). The excess CHD standardised mortality ratio (SMR) compared to the population in England and Wales was calculated (with 95% Confidence intervals). Results: There were 252 deaths from CHD. Overall, treated DFH patients had a higher CHD SMR than PFH patients (post 1991 35% higher 2.40 (2.00-2.86) vs 1.78 (1.44-2.19) p = 0.03). In treated DFH patients with previous CHD the CHD SMR was significantly elevated at all time periods but in men fell 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 these values were 7.23 (2.65-15.73), 4.42 (2.70-6.82) and 6.34 (2.06-14.81)). In treated DFH men with no previous CHD the CHD SMR fell over the three time periods, and was not significantly elevated post 2008 (0.89 (0.29-2.08), but in women the SMR remained significantly elevated (post 2008 3.65 (1.75-6.72)). Conclusions: The data confirm the major benefit in CHD mortality associated with statin treatment, but suggest that FH patients with pre-existing disease, and women with FH may not be being treated adequately.