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MRI assessment of correlation between cardiac biventricular function, myocardial iron overload and pancreatic iron overload in a large cohort of thalassemia major patients
© Restaino et al; licensee BioMed Central Ltd. 2010
Published: 21 January 2010
β-Thalassaemia major is a hereditary anaemia characterized by ineffective erythropoiesis and haemolysis. By the age of 3 months, severe anaemia develops leading to increased intestinal iron absorption. To maintain haemoglobin at acceptable levels, patients need to be given repeated blood transfusions. A major drawback of this treatment is transfusional siderosis, which, in association other mechanisms, leads to iron overload. The iron accumulates primarily in the liver and spleen, later on in the heart and the endocrine glands. Increased iron deposition is cytotoxic and may cause organ dysfunction. The leading cause of death in these patients is cardiac failure, but impairment of the endocrine and exocrine function of the pancreas is a common complication.
The use of non-invasive techniques for monitoring iron overload in the affected organs is advisable. Multiecho T2* MRI is a well established technique for heart and liver iron overload assessment. There are few reports on the use of MRI to study iron deposits in the pancreas and on the correlation between pancreatic siderosis and myocardial iron overload and function.
To describe the T2* values of the pancreas in patients with beta-thalassemia major; to investigate the correlation between pancreatic and myocardial siderosis; to investigate the correlation between pancreatic iron overload and biventricular cardiac function.
Study population: 147 consecutive thalassemia major patients enrolled in the Myocardial Iron Overload in Thalassemia network, an Italian network constituted by thalassemia and MRI centers. MRI was performed using a 1.5-T MRI scanner. Myocardial T2* was measured with a fast-gradient-echo multi-echo sequence with ECG triggering. Biventricular function was quantitatively evaluated with a steady-state free procession cines acquired during 10 s breath holds in sequential 8 mm short axis slices from the atrio-ventricular ring to the apex. Pancreatic T2* was measured with a gradient-echo multiecho sequence. Analysis on T2* images was performed with a dedicated software.
Pancreatic iron overload is positively correlated to myocardial iron overload and negatively correlated to biventricular systolic function.
This article is published under license to BioMed Central Ltd.