Initially, statins with ezetimibe are introduced and in responsive patients also PCSK9 inhibitors, but frequently do not result in a satisfactory reductions of either TC or LDL-C levels, especially in moderate and severe HoFH patients, which have the highest risk of CVD (Kolansky et al., 2008; Rajendran et al., 2013). This evidence concerns the gene PCSK9 and homozygous familial hypercholesterolemia.