Presentation
Hereditary spherocytosis with multiple blood transfusions
Patient Data
Evidence of severe myocardial and mild hepatic iron overload on T2*/R2* mapping:
- Myocardium: T2* = 4 ms, R2* = 251 Hz, dry weight >/=2.7 mg/g
- Liver: T2* = 9.4 ms, R2* = 106 Hz, dry weight = 2-7 mg/g
Mildly dilated LV cavity with mildly impaired global LV systolic function (EF=52%).
No evidence of late gadolinium enhancement (LGE) of the myocardium to suggest fibrosis or infarction.
Mild left atrial dilatation.
The above findings are consistent with iron overload cardiomyopathy.
Hepatomegaly.
Splenomegaly.
Diffuse hypointensity of the liver parenchyma and bone marrow consistent with changes from secondary iron overload.
Case Discussion
Iron overload cardiomyopathy can result from secondary iron overload primarily associated with transfusion dependent anemias such as thalassemia, which can progress to dilated cardiomyopathy with chamber dilatation and impaired systolic function.
Cardiac MRI with T2* can quantify myocardial and hepatic iron content with resulting T2* (ms) and R2* (Hz) values and corresponding tissue iron levels in mg/g dry weight. The severity of myocardial iron overload and therefore the T2* value can predict the risk of developing heart failure with a threshold value of 20 ms with T2* values <10 ms being the highest risk.
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