Westra WH, Hruban RH, Baughman KL, Olson JL, Porterfield JK, Mitchell MC, Hutchins GM Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, Maryland.
Dilated cardiomyopathy is a frequent and serious complication of idiopathic hemochromatosis.
The mechanism by which disordered iron metabolism induces heart failure is not entirely understood, but myocardial dysfunction appears to be intimately related to the deposition of iron in myocytes. Cardiac function characteristically worsens or improves in proportion to the degree of iron accumulation in cardiac myocytes. An initial endomyocardial heart biopsy demonstrated severe iron deposition in myocytes. The patient's heart failure worsened in the next 3 years and he eventually required a heart transplant. Examination of the explanted heart revealed dilated cardiomyopathy, but the previously demonstrated iron deposits in the cardiac myocytes were depleted.