IJCS | Volume 31, Nº3, May/ June 2018

309 Figure 1 – Echocardiogram by Strain method: First image shows left ventricular systolic dysfunction demonstrated by the value of -9.8 obtained by the global LV Strain. Second image performed after treatment shows recovery of LV systolic function demonstrated by the Strain value of -20.8. With the suspicion of heart disease caused by iron deposition due to the results of laboratory tests and physical examination, complementary tests were performed to close the diagnosis. Magnetic resonance imaging by the T2* methodwas suggestive of myocardial and hepatic iron deposition (Figure 1). The pre-treatment cardiac MRI showed a time of T2* 13.0 ms (N: > 20 ms) and an estimatedMIC (Miocardial Iron Concentration) of 2.0 mg/g (N: < 1.1 mg/g). After treatment the time of T2* was 17.0 ms, withMIC estimated at 1.3 mg/g. This result was confirmed by endomyocardial biopsy. Research continued with the exclusion of secondary causes of hemochromatosis and the genetic analysis that excluded HH related to the HFE gene. In conjunction with the clinical data the case is suggestive of Juvenile Hemochromatosis. The established treatment basedonweeklyphlebotomies in association with the use of oral and parenteral iron chelators, aswell asconventional beta‑blocker,ACEinhibitor, spironolactone and furosemide therapy. After 6 months of outpatient follow-up, the patient presented improvement of the functional capacity and improvement of the echocardiographic parameters (figure 2). Discussion The main diagnostic hypothesis is non-classical hemochromatosis, or not linked to the HFE gene, the juvenile type being the most compatible with the clinical picture presented. Juvenile HH is characterized by early accumulation of iron in the body, with manifestations between the 2nd and 3rd decades of life. The manifestations include hypogonadotrophic hypogonadism, heart disease, cirrhosis, diabetes, arthropathy and skin pigmentation. It is characterized by rapid accumulation of iron in the body, early onset, with manifestations of iron overload between the second and third decades of life (15-20 years of age) and functional impairment of affected organs before 30 years of age. Cardiac manifestations with heart failure and arrhythmias are early and are important causes of death. 6 Cardiomyopathy due to iron overload, whether caused by hemochromatosis or not, is a disease that should always be considered as soon as begins the diagnosis of patients with heart failure. The patient described abovemanifested the diseasewith a classic picture of congestive heart failure and already presented a significant irondeposition burden on the heart. Although the disease was no longer at an early stage, the natural history of the diseasewasmodified and there was regression of iron accumulation as well as improvement of ventricular function. It is worth mentioning that cardiac MRI using the T2* methodwas established as a diagnosticmethod aswell as a method for risk stratification in these patients. It can also be used to follow the response to the treatment of the disease. 7 Iron overload cardiomyopathy is a potentially lethal but treatable disease when diagnosed and treated early in its course. Despite the low incidence of heart disease associated with iron overload in the general population, it is a cardiopathy with potential for treatment and reversal, and its screening tests are easy to perform and inexpensive. Therefore, iron, ferritin and transferrin saturation should be part of the initial propaedeutic routine of patients with dilated cardiomyopathy. Iglesias et al. Hemochromatosis: A reversible cause of heart failure International Journal of Cardiovascular Sciences. 2018;31(3)308-311 Case Report