Abstract
Iron overload cardiomyopathy (IOC) is a secondary form of cardiomyopathy occurring in the setting of iron accumulation in the myocardium, mainly because of genetically determined disorders of iron metabolism (primary hemochromatosis) or multiple transfusions (secondary hemochromatosis). Two phenotypes of IOC have been identified: the dilated phenotype and the restrictive phenotype, with the former being more frequent. Cardiac magnetic resonance (CMR) has radically changed the management of patients with hemochromatosis. The T2* technique is currently the mainstay for the noninvasive quantification of myocardial iron overload (MIO) and for the design of tailor-made chelation therapies and the evaluation of their efficacy. The widespread use in clinical practice of the T2* technique contributed to open the prognosis of patients with hemochromatosis. Moreover, T1 mapping was demonstrated able to identify even small amounts of iron accumulated in the heart. Therefore, the use of both T1 and T2* mapping has been proposed as a valid means to improve the detection of mild or early MIO.
Furthermore, in hemochromatosis a multiparametric CMR approach combining the MIO quantification with tissue characterization for fibrosis and edema and with morphological and functional parameters is strongly recommended. In hemochromatosis, replacement myocardial fibrosis detected by the late gadolinium enhancement technique has emerged as a strong CMR predictor for cardiac complications and an increased extracellular volume (ECV) fraction has been demonstrated, suggesting the presence of diffuse myocardial fibrosis. In addition, the iron-induced oxidative stress and the increased risk of infections in transfused patients can trigger myocardial inflammation that can be detected by T2 mapping. The assessment of biventricular volumes and function by cine images, for which CMR is the gold standard, is crucial for the detection of heart damage, especially on the basis of the consistent number of patients showing heart dysfunction without evidence of MIO. The routinely acquired cine images can also be used for detecting the morphological criteria for non-compaction and for quantifying the myocardial deformation by ventricular strain feature tracking (FT) CMR.
In the near future, both FT-CMR and the evaluation for ECV can help in identifying treatment strategies able to bridge the gap between the onset of subclinical alterations and overt heart failure.
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Short-axis cine stack of the follow-up scan (MP4 174 kb)
Short-axis cine stack (MP4 180 kb)
Four-chamber cine SSFP (MP4 187 kb)
Four-chamber SSFP cine view (MP4 172 kb)
Feature tracking strain analysis in a 4-chamber cine view (MP4 228 kb)
Short-axis cine stack (MP4 545 kb)
Short-axis cine stack (MP4 194 kb)
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Pepe, A., Sormani, P., Meloni, A., Torlasco, C. (2023). Iron Overload Cardiomyopathies. In: Barison, A., Dellegrottaglie, S., Pontone, G., Indolfi, C. (eds) Case-based Atlas of Cardiovascular Magnetic Resonance. Springer, Cham. https://doi.org/10.1007/978-3-031-32593-9_10
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