The heart does not accumulate iron disproportionately to other organs. The key to the heart s central role in the pathology of iron overload lies in the need for the complex array of cells and structures in the heart to function coordinately. Half of the liver can be lost to fibrosis or cirrhosis, and a person can still survive. This is not true of the heart. As importantly, the consequences of disrupted signal transduction between the AV node and the ventricles can be catastrophic and even fatal.
Hereditary hemochromatosis reflects a fractional increase in dietary iron absorption.
Tissue iron reaches dangerous levels after thirty or forty years. The gene responsible for hereditary hemochromatosis, HFE, resides on chromosome 6. I HLA antigens. People with two copies of the mutant protein can develop iron overload and potentially a myriad of problems. Only recently have investigators gained insight into the mechanism by which the mutation in HFE alters cellular iron metabolism.