Management of cardiac hemochromatosis

Archives of Medical Science : AMS
Wilbert S Aronow

Abstract

Iron-overload syndromes may be hereditary or acquired. Patients 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. Cardiac hemochromatosis should be considered in any patient with unexplained heart failure. Screening for systemic iron overload with serum ferritin and transferin saturation should be performed. If these tests are consistent with iron overload, further noninvasive and histologic confirmation is indicated to confirm organ involvement with iron overload. Cardiac magnetic resonance imaging is superior to other diagnostic tests since it can quantitatively assess myocardial iron load. Therapeutic phlebotomy is the therapy of choice in nonanemic patients with cardiac hemochromatosis. Therapeutic phlebotomy should be started in men with serum ferritin levels of 300 μg/l or more and in women with serum ferritin levels of 200 μg/l or more. Therapeutic phlebotomy consists of removing 1 unit of blood (450 to 500 ml) ...Continue Reading

Citations

Jan 23, 2020·AJR. American Journal of Roentgenology·Elizabeth LeeJadranka Stojanovska
Nov 2, 2019·Panminerva Medica·Michele CorrealeNatale D Brunetti
Apr 19, 2020·European Radiology Experimental·E E NazarovaG A Novichkova
Mar 5, 2020·European Heart Journal. Case Reports·Vaida SudmantaitėRimgaudas Katkus

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