The influence of PCSK6 on the cardiovascular system and disease development still remains largely unknown, although variants in the PCSK6 genomic locus have been associated with congenital heart disease and aortic dissection [26,27] and PCSK6 has been implicated as a regulator of blood pressure in mice subjected to a sodium chloride enriched diet [28]. This evidence concerns the gene PCSK6 and congenital heart disease.