Reductions in all-cause, cancer, and coronary mortality in statin-treated patients with heterozygous familial hypercholesterolaemia: a prospective registry study

Abstract

AimsTo examine the changes in coronary, all-cause, and cancer mortality in patients with heterozygous familial hypercholesterolaemia (FH) before and after lipid-lowering therapy with statins.Methods and resultsA total of 3382 patients (1650 men) aged <80 years were recruited from 21 lipid clinics in the United Kingdom and followed prospectively between 1980 and 2006 for 46 580 person-years. There were 370 deaths, including 190 from coronary heart disease (CHD) and 90 from cancer. The standardized mortality ratio (compared with the population in England and Wales) was calculated before and from 1 January 1992. In patients aged 20–79 years, CHD mortality fell significantly by 37% (95% CI = 7–56) from 3.4- to 2.1-fold excess. Primary prevention resulted in a 48% reduction in CHD mortality from 2.0-fold excess to none, with a smaller reduction of nearly 25% in patients with established disease. Coronary mortality was reduced more in women than in men. In patients without known CHD at registration, all-cause mortality from 1992 was 33% (21–43), lower than in the general population, mainly due to a 37% (21–50) lower risk of fatal cancer.ConclusionThe results emphasize the importance of early identification of FH and treatment with statins.

Integrated guidance on the care of familial hypercholesterolaemia from the International FH Foundation

“…Statins reduce the risk for cardiovascular events [108][109][110][111][112][113] and progression of atherosclerosis in FH [56], and their use is cost-effective [77,114,115]. Adjuvant therapy with ezetimibe, bile acid sequestrants, plant stanols/sterols, and niacin (or its derivatives) may often also be required [21,[116][117][118][119][120].…”

“…As in adults, good evidence for an absolute or relative target does not exist in children; international guidelines are consequently not uniform in their recommendations [8,9,19,20,22,65]. Early cholesterol-lowering treatment can substantially alter the natural history of FH [53,56,[108][109][110][111][112]145]. Although systematic reviews confirm the safety of currently employed statins in children [146,147], the long-term sequelae of high intensity statin regimens are unknown.…”

Familial hypercholesterolemia: The Italian Atherosclerosis Society Network (LIPIGEN)

“…In fact, in line with coronary heart disease (CHD) mortality rates reported in heterozygous FH (HeFH) patients from the UK [6], the risk for CHD was increased by almost four-fold in HeFH patients in the Netherlands [7] compared to unaffected relatives.…”

“…Yet, of the expected 66,800 HeFH patients in the Netherlands based on the estimated prevalence of HeFH of 1:250 [6], only 38.6% were diagnosed after 20 years of screening, underlying that policy makers and health care professionals need to be made more strongly aware of the urgency of identification of FH patients.…”

Improved access to life insurance after genetic diagnosis of familial hypercholesterolaemia: cross-sectional postal questionnaire study

“…7 Since then, several findings have been published, which show that the prognosis for treated FH patients has improved in the post statin era. 5,12 These findings may have influenced decision-making among insurers underwriting life insurance even further. Thus, whether improvement in acceptance rate over time is owing to the guidelines or to awareness of improved prognosis of treated FH patients remains difficult to determine.…”