What is it about?
Average life expectancy in untreated homozygous familial hypercholesterolaemia (FH) is only 18 due to premature death from severe atherosclerosis of the aortic root and coronary arteries. Our retrospective analysis of 133 FH homozygotes followed up for 25 years in South Africa and the UK revealed that those with a serum cholesterol of >15.1 mmol/l had a hazard ratio for all cause mortality that was 11.5 times that of those with serum cholesterol <8.1 mmol/l. Lowest mortality rates were seen in patients treated with a combination of apheresis, statins and ezetimibe. Additional benefit can be expected from the use of newer lipid-lowering agents such as evolocumab and lomitapide.
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Why is it important?
This is the first study to document the graded effects of lipid-lowering therapy on outcome in homozygous FH, a notoriously difficult disorder to treat
Perspectives
This final peer-reviewed publication of my career vindicates the introduction by myself and colleagues in 1975 of long-term apheresis to treat homozygous FH.
Gilbert Thompson
Imperial College London
Read the Original
This page is a summary of: Survival in homozygous familial hypercholesterolaemia is determined by the on-treatment level of serum cholesterol, European Heart Journal, July 2017, Oxford University Press (OUP),
DOI: 10.1093/eurheartj/ehx317.
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