Cardiology

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

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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)

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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)

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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.

HEART UK statement on the management of homozygous familialhypercholesterolaemia in the United Kingdom (2016)

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Type of publication:
Journal article

Author(s):
France M., Rees A., Datta D., Thompson G., *Capps N., Ferns G., Ramaswami U., Seed M., Neely D., Cramb R., Shoulders C., Barbir M., Pottle A., Eatough R., Martin S., Bayly G., Simpson B., Halcox J., Edwards R., Main L., Payne J., Soran H.

Citation:
Atherosclerosis, December 2016, vol./is. 255/(128-139)

Abstract:
This consensus statement addresses the current three main modalities of treatment of homozygous familial hypercholesterolaemia (HoFH): pharmacotherapy, lipoprotein (Lp) apheresis and liver transplantation. HoFH may cause very premature atheromatous arterial disease and death, despite treatment with Lp apheresis combined with statin, ezetimibe and bile acid sequestrants. Two new classes of drug, effective in lowering cholesterol in HoFH, are now licensed in the United Kingdom. Lomitapide is restricted to use in HoFH but, may cause fatty liver and is very expensive. PCSK9 inhibitors are quite effective in receptor defective HoFH, are safe and are less expensive. Lower treatment targets for lipid lowering in HoFH, in line with those for the general FH population, have been proposed to improve cardiovascular outcomes. HEART UK presents a strategy combining Lp apheresis with pharmacological treatment to achieve these targets in the United Kingdom (UK). Improved provision of Lp apheresis by use of existing infrastructure for extracorporeal treatments such as renal dialysis is promoted. The clinical management of adults and children with HoFH including advice on pregnancy and contraception are addressed. A premise of the HEART UK strategy is that the risk of early use of drug treatments beyond their licensed age restriction may be balanced against risks of liver transplantation or ineffective treatment in severely affected patients. This may be of interest beyond the UK.

Comparison of the size of persistent foramen ovale and atrial septal defects in divers with shunt-related decompression illness and in the general population (2015)

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Type of publication:
Journal article

Author(s):
Wilmshurst P.T., Morrison W.L., Walsh K.P., Pearson M.J., Nightingale S.

Citation:
Diving and Hyperbaric Medicine, June 2015, vol./is. 45/2(89-93)

Abstract:
Introduction: Decompression illness (DCI) is associated with a right-to-left shunt, such as persistent foramen ovale (PFO), atrial septal defect (ASD) and pulmonary arteriovenous malformations. About one-quarter of the population have a PFO, but considerably less than one-quarter of divers suffer DCI. Our aim was to determine whether shunt-related DCI occurs mainly or entirely in divers with the largest diameter atrial defects. Methods: Case control comparison of diameters of atrial defects (PFO and ASD) in 200 consecutive divers who had transcatheter closure of an atrial defect following shunt-related DCI and in an historic group of 263 individuals in whom PFO diameter was measured at post-mortem examination. Results: In the divers who had experienced DCI, the median atrial defect diameter was 10 mm and the mean (standard deviation) was 9.9 (3.6) mm. Among those in the general population who had a PFO, the median diameter was 5 mm and mean was 4.9 (2.6) mm. The difference between the two groups was highly significant (P < 0.0001). Of divers with shuntrelated DCI, 101 (50.5%) had an atrial defect 10 mm diameter or larger, but only 1.3% of the general population studied had a PFO that was 10 mm diameter of larger. Conclusions: The risk of a diver suffering DCI is related to the size of the atrial defect rather than just the presence of a defect.

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