Haemochromatosis (cardiac manifestations)
Cardiac involvement in haemochromatosis typically occurs with primary haemochromatosis, as the organ is usually spared in the secondary form of the disease.
Cardiac involvement occurs in approximately 15-20% of the patients with haemochromatosis.
Manifestations depend on the extent of iron deposition and include:
- pedal oedema
- features of congestive heart failure
Haemochromatosis can result in an iron overload cardiomyopathy.
Findings are nonspecific but may show a decrease in systolic function and/or ejection fraction. May also show evidence of a dilated cardiomyopathy.
The role of cardiac MRI (CMR) is to identify and quantify the amount of iron deposition. Paramagnetic ferritin and haemosiderin lead to altered relaxation times of adjacent hydrogen nuclei. T2* imaging is highly sensitive in detecting the amount of iron deposition. T2* CMR is also a recent advancement in the evaluation of cardiac iron overload.
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