ArticleJournal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance2013
On myocardial siderosis and left ventricular dysfunction in hemochromatosis.
Article in Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 40 citations in OpenAlex.
- Position Statement on Multimodal Cardiac Imaging in Patients with Systemic Diseases - 2026.Arquivos brasileiros de cardiologia · 2026Article
- Utilities and Limitations of Cardiac Magnetic Resonance Imaging in Dilated Cardiomyopathy.Korean journal of radiology · 2023Review
- Left Ventricular Function and Iron Loading Status in a Tertiary Center Hemochromatosis Cohort-A Cardiac Magnetic Resonance Study.Diagnostics (Basel, Switzerland) · 2022Article
- Pathogenesis, Diagnosis, and Clinical Implications of Hereditary Hemochromatosis-The Cardiological Point of View.Diagnostics (Basel, Switzerland) · 2021Review
- Improvements in cardiac function detected using echocardiography in patients with hereditary haemochromatosis.Irish journal of medical science · 2020Article
- Difficult diagnosis of cardiac haemochromatosis: a case report.European heart journal. Case reports · 2020Article
- Balance of cardiac and systemic hepcidin and its role in heart physiology and pathology.Laboratory investigation; a journal of technical methods and pathology · 2018Review
- Review of Journal of Cardiovascular Magnetic Resonance 2014.Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance · 2015Review
- SLC39A14 Is Required for the Development of Hepatocellular Iron Overload in Murine Models of Hereditary Hemochromatosis.Cell metabolism · 2015Article
- Review
- Cardiac magnetic resonance in clinical cardiology.World journal of cardiology · 2015Review
- Review of Journal of Cardiovascular Magnetic Resonance 2013.Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance · 2014Review
- High ferritin levels have major effects on the morphology of erythrocytes in Alzheimer's disease.Frontiers in aging neuroscience · 2013Article
- Upregulation of rat P23 (a member of the YjgF protein family) by fasting, glucose diet and fatty acid feeding.Cellular and molecular life sciences : CMLS · 2004Article
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Authors and funding
6 authors at 5 institutions in 1 country.
Funding
Abstract
backgroundChronically increased intestinal iron uptake in genetic hemochromatosis (HC) may cause organ failure. Whilst iron loading from blood transfusions may cause dilated cardiomyopathy in conditions such as thalassemia, the in-vivo prevalence of myocardial siderosis in HC is unclear, and its relation to left ventricular (LV) dysfunction is controversial. Most previous data on myocardial siderosis in HC has come from post-mortem studies.
methodsCardiovascular magnetic resonance (CMR) was performed at first presentation of 41 HC patients (58.9 ± 14.1 years) to measure myocardial iron and left ventricular (LV) ejection fraction (EF).
resultsIn 31 patients (genetically confirmed HFE-HC), the HFE genotype was C282Y/C282Y (n = 30) and C282Y/H63D (n = 1). Patients with other genotypes (n = 10) were labeled genetically unconfirmed HC. Of the genetically confirmed HFE-HC patients, 6 (19%) had myocardial siderosis (T2* <20 ms). Of these, 5 (83%) had heart failure and reduced LVEF which was correlated to the severity of siderosis (R2 0.57, p = 0.049). Two patients had follow-up scans and both had marked improvements in T2* and LVEF following venesection. Myocardial siderosis was present in 6/18 (33%) of patients with presenting ferritin ≥ 1000 μg/L at diagnosis but in 0/13 (0%) patients with ferritin <1000 μg/L (p = 0.028). Overall however, the relation between myocardial siderosis and ferritin was weak (R2 0.20, p = 0.011). In the 10 genetically unconfirmed HC patients, 1 patient had mild myocardial siderosis but normal EF. Of all 31 patients, 4 had low LVEF from other identifiable causes without myocardial siderosis.
conclusionMyocardial siderosis was present in 33% of newly presenting genetically confirmed HFE-HC patients with ferritin >1000 μg/L, and was the commonest cause of reduced LVEF. Heart failure due to myocardial siderosis was only found in these HFE-HC patients, and was reversible with venesection. Myocardial iron was normal in patients with other causes of LV dysfunction.
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