Contribution of Cardiac Magnetic Resonance to Hematochromatosis

Abstract

Hemochromatosis is a disease characterized by the progressive accumulation of iron in the body. It can be primitive or secondary. It affects several organs including the heart, liver, pancreas and pituitary gland. The preponderant cardiac involvement is myocardial, related to the iron myocyte overload, causing a decrease in left ventricular distensibility. Echocardiography is the first-line examination, showing an abnormality of left ventricular filling and, later, cavitary dilatation with left ventricular systolic dysfunction. Cardiac MRI is the gold standard for measuring excess iron in the myocardium in the context of hemochromatosis using the T2 * technique. It plays a major role for the diagnosis of cardiac involvement and for monitoring the chelation treatment. In this study, we report three cases of patients with hemochromatosis whose cardiac localization was confirmed or eliminated using cardiac MRI. Cardiovascular magnetic resonance has been used to assess myocardial iron deposition using the relaxation parameters T2*. Heart T2* falls with increasing iron loading. One patient had a myocardial T2* below 20 ms indicating myocardial siderosis. The others patients had a T2 *> 20 ms. The late enhancement was absent in all our patients. This study aims at detecting the myocardial iron overload using cardiac MRI. The treatment is based on bleeding in primary hemochromatosis while in secondary hemochromatosis, it is based on excretion of iron by chemical chelation.