Hemochromatosis (cardiac manifestations)
Updates to Article Attributes
Cardiac involvement in haemochromatosis typically occurs with primary haemochromatosis, as the organ is usually spared in the secondary form of the disease.
Epidemiology
Cardiac involvement occurs in approximately 15-20% of the patients with haemochromatosis.
Clinical presentation
Manifestations depend on the extent of iron deposition and include:
- breathlessness
- pedal oedema
- palpitations
- features of congestive heart failure
Pathology
Haemochromatosis can result in an ironiron overload cardiomyopathy.
Radiographic features
Echocardiography
Findings are nonspecific but may show a decrease in systolic function and/or ejection fraction. May also show evidence of a dilated cardiomyopathy.
MRI
RoleThe role of cardiac MRI (CMR) is to identify and quantify the amount of iron deposition. Paramagnetic ferritin and haemosiderin leadslead 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.
-</ul><h4>Pathology</h4><p>Haemochromatosis can result in an<a href="/articles/iron-overload-cardiomyopathy"> iron overload cardiomyopathy</a>.</p><h4>Radiographic features </h4><h5>Echocardiography</h5><p>Findings are nonspecific but may show a decrease in systolic function and/or ejection fraction. May also show evidence of a dilated cardiomyopathy.</p><h5>MRI</h5><p>Role of MRI is to identify and quantify the amount of iron deposition. Paramagnetic ferritin and haemosiderin leads to altered relaxation times of adjacent hydrogen nuclei. <a href="/articles/t2-relaxation-1">T2* imaging</a> is highly sensitive in detecting the amount of iron deposition. T2* CMR is also a recent advancement in evaluation of cardiac iron overload.</p>- +</ul><h4>Pathology</h4><p>Haemochromatosis can result in an <a href="/articles/iron-overload-cardiomyopathy">iron overload cardiomyopathy</a>.</p><h4>Radiographic features </h4><h5>Echocardiography</h5><p>Findings are nonspecific but may show a decrease in systolic function and/or ejection fraction. May also show evidence of a dilated cardiomyopathy.</p><h5>MRI</h5><p>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. <a href="/articles/t2-relaxation-1">T2* imaging</a> is highly sensitive in detecting the amount of iron deposition. T2* CMR is also a recent advancement in the evaluation of cardiac iron overload.</p>
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