Iron Chelation Therapy for Treatment of Cardiac Hemochromatosis

Hemochromatosis is a clinical syndrome caused by abnormal accumulation of iron in parenchymal organs leading to organ toxicity and dysfunction. Cardiac hemochromatosis is a cardiomyopathy due to primary iron-overload cardiomyopathy which causes congestive heart failure. Patients with cardiac hemochromatosis may be asymptomatic early in the disease. Once heart failure develops, there is rapid deterioration. Cardiac hemochromatosis is characterized by a dilated cardiomyopathy with dilated ventricles, reduced ejection fraction, and reduced fractional shortening. Deposition of iron may occur in the entire cardiac conduction system, especially the atrioventricular node [1]. Cardiac hemochromatosis should be ruled out in patients with unexplained congestive heart failure.