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Eur Heart J. 2018 Apr 7;39(14):1162-1168. doi: 10.1093/eurheartj/ehx317.

Survival in homozygous familial hypercholesterolaemia is determined by the on-treatment level of serum cholesterol.

Author information

1
Department of Metabolic Medicine, Faculty of Medicine, Imperial College London, Hammersmith Campus, Ducane Road, London W12?0NN, UK.
2
Division of Lipidology, Department of Medicine, UCT Faculty Health Sciences, University of Cape Town, Anzio Road, 7925 Observatory, Cape Town, South Africa.
3
Division of Chemical Pathology, Department of Pathology, UCT Faculty Health Sciences, University of Cape Town, Anzio Road, 7925 Observatory, Cape Town, South Africa.
4
Department of Cardiology, Imperial College Healthcare NHS Trust, Charing Cross Hospital, Fulham Palace Road, London W6 8RF, UK.
5
Carbohydrate and Lipid Metabolism Research Unit, Department of Medicine, University of the Witwatersrand, 7 York Rd, Parktown 2193, Johannesburg, South Africa.

Abstract

Aims:

Homozygous familial hypercholesterolaemia (FH) is a rare inherited disorder characterized by extreme hypercholesterolaemia from birth, accelerated atherosclerosis, and premature death. Many forms of lipid-lowering therapies have been used in the past, but definitive evidence of benefit has been lacking. We therefore undertook a retrospective survey of lipid levels and clinical outcomes of FH homozygotes treated with a combination of lipid-lowering measures between 1990 and 2014 in South Africa and the UK.

Methods and results:

We divided 133 previously statin-naive homozygotes into quartiles according to their on-treatment levels of serum cholesterol and compared the occurrence of any death, cardiovascular death, and major adverse cardiovascular events (MACE) between the quartiles during 25 years of follow-up, using Cox and competing risks regression analysis. Patients in Quartile 4, with an on-treatment serum cholesterol >15.1 mmol/L, had a hazard ratio of 11.5 for any death compared with those in Quartile 1, with an on-treatment cholesterol of < 8.1 mmol/L. Those in Quartiles 2 and 3 combined, with on-treatment cholesterol of 8.1-15.1 mmol/L had a hazard ratio of 3.6 compared with Quartile 1. These differences were statistically significant (P < 0.001) and remained so after adjustments for confounding factors (P = 0.04). Significant differences between quartiles were also evident for cardiovascular deaths and MACE.

Conclusion:

These findings provide unequivocal evidence that the extent of reduction of serum cholesterol achieved by a combination of therapeutic measures, including statins, ezetimibe, lipoprotein apheresis, and evolocumab, is a major determinant of survival in homozygous FH.

PMID:
29106543
DOI:
10.1093/eurheartj/ehx317

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