DIAGNOSIS AND MANAGEMENT OF CARDIAC HEMOCHROMATOSIS F Shariati MD, M Sultan MD, H Field MD. Department of Medicine, Ochsner Medical Center, New Orleans, LA
INTRODUCTION Cardiac hemochromatosis is an important and preventable cause of heart failure. It often starts in adults ages 30-50 with hiddenprogression tocardiacdysfunction. It is characterizedbydilatedcardiomyopathywithdilatedventricles, and reduced ejection fraction. Unfortunately, once heart failure develops, there is a rapid deterioration. Therapeutic phlebotomy is the treatment of choice in nonanemic patients with cardiac hemochromatosis. CASE: A51-year-old-manwas admittedwith acutedecompensatedheart failure andnewonset atrial fibrillation. He had a history of hereditary hemochromatosis, however, was non-compliant with medical follow up and with phlebotomy. On examination he appeared to have bronze skin and yellow eyes along with significant peripheral edema. Iron metabolism markers were obtained and were highly elevated. Echocardiography was significant for dilated ventricles, reduced ejection fraction, and reduced fractional shortening. Cardiac magnetic resonance (CMR) was consistent with diagnosis of myocardial siderosis. Standard heart failure treatment along with chelation therapy and regular phlebotomies were initiated. DISCUSSION: Cardiac hemochromatosis should be considered in any patient with new onset heart failure and treatment should be started in a timelymanner as if left untreated the average survival is less than a year in thosewith severe cardiac impairment. However, if diagnosed and treated early, the survival rate can approach that of the regular heart failure population.
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