A case of iron overload cardiomyopathy: beneficial effects of iron chelating agent and calcium channel blocker on left ventricular dysfunction.

We experienced an 81 year-old man with heart failure and macrocytic anemia. His serum ferritin level was extremely high (> 3,000 ng/mL). Echocardiography showed a normal left ventricular (LV) ejection fraction (EF), although the total ejection isovolume index (TEI index) was markedly elevated (0.60). In a cardiac catheterization study, cardiac index, pulmonary arterial wedge pressure, LV wall motion, and coronary arteries were shown to be normal. However, atrial pacing demonstrated a negative force-frequency relationship (a decrease in arterial blood pressure with higher pacing rates). Pathological study showed hemosiderin accumulation in his liver, but not in his myocardial tissue. As earlier studies have reported that iron may play an important role in oxidative cell damage and that this ion can enter cardiomyocytes through L-type Ca(2+) channels, we started an iron chelating agent (deferoxamine) and a calcium channel blocker (verapamil) in this case. Eighteen months later, his serum ferritin levels fell significantly without any changes in anemia. The TEI index was normalized (0.21) and the atrial pacing provoked a less negative force-frequency relationship. Thus, this combination treatment may be effective in iron overload cardiomyopathy at its early stage, when LV diastolic dysfunction is dominant and LV systolic dysfunction is only latent.