CHF typically occurs in patients who also have manifestations in other organs, particularly the kidneys, as seen in autosomal recessive polycystic kidney disease (ARPKD), which is the most known and studied of this group, due to mutations in the PKHD1 gene (encodes fibrocystin located on the membrane of the cilia), generating a mutated polycistin 2 and a calcium channel, thus increasing the amount of intracellular calcium and cAMP [95]. Here, PKHD1 is linked to autosomal recessive polycystic kidney disease.