Cardiomyopathy in hemochromatosis

Changed by Yuranga Weerakkody, 29 Nov 2014

Updates to Synonym Attributes

Updates to Article Attributes

Body was changed:

Cardiomyopathy in haemochromatosis refers to an iron overload cardiomyopathy which can occur in those with the condition. Please refer

In primary haemochromatosis leading to that article for imaging featuresiron overload, the cardiomyopathy has classically categorised as an infiltrative cause of restrictive cardiomyopathy. While in those with secondary haemochromatosis may lead to a dilated cardiomyopathy with severe diastolic LV dysfunction in the early stages of the disease, before LVEF is affected 2.

Practical points

The H63D mutation (common mutation of the haemochromatosis associated gene) has been shown to be significantly increased in patients with idiopathic dilated cardiomyopathy although these patients to do not get classified as a cardiomyopathy in haemochromatosis unless there is an iron overload cardiomyopathy 3.

See also

  • -<p><strong>Cardiomyopathy in haemochromatosis</strong> refers to an <a title="iron overload cardiomyopathy" href="/articles/iron-overload-cardiomyopathy">iron overload cardiomyopathy</a>. Please refer to that article for imaging features.</p><h4>See also</h4><ul>
  • -<li><a title="Haemochromatosis" href="/articles/haemochromatosis">haemochromatosis</a></li>
  • -<li><a title="Cardiomyopathies" href="/articles/cardiomyopathy-1">cardiomyopathy</a></li>
  • +<p><strong>Cardiomyopathy in haemochromatosis</strong> refers to an <a href="/articles/iron-overload-cardiomyopathy">iron overload cardiomyopathy</a> which can occur in those with the condition.</p><p>In primary haemochromatosis leading to iron overload, the cardiomyopathy has classically categorised as an infiltrative cause of restrictive cardiomyopathy. While in those with secondary haemochromatosis may lead to a dilated cardiomyopathy with severe diastolic LV dysfunction in the early stages of the disease, before LVEF is affected <sup>2</sup>.</p><h4>Practical points</h4><p>The H63D mutation (common mutation of the haemochromatosis associated gene) has been shown to be significantly increased in patients with <a href="/articles/idiopathic-dilated-cardiomyopathy">idiopathic dilated cardiomyopathy</a> although these patients to do not get classified as a cardiomyopathy in haemochromatosis unless there is an iron overload cardiomyopathy <sup>3</sup>.</p><h4>See also</h4><ul>
  • +<li>
  • +<a href="/articles/haemochromatosis">haemochromatosis</a><ul><li><a href="/articles/cardiac-manifestations-of-haemochromatosis">cardiac manifestations of haemochromatosis</a></li></ul>
  • +</li>
  • +<li><a href="/articles/cardiomyopathy-1">cardiomyopathy</a></li>

References changed:

  • 1. Murphy CJ, Oudit GY. Iron-overload cardiomyopathy: pathophysiology, diagnosis, and treatment. J. Card. Fail. 2010;16 (11): 888-900. <a href="http://dx.doi.org/10.1016/j.cardfail.2010.05.009">doi:10.1016/j.cardfail.2010.05.009</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/21055653">Pubmed citation</a><span class="auto"></span>
  • 3. Mahon NG, Coonar AS, Jeffery S et-al. Haemochromatosis gene mutations in idiopathic dilated cardiomyopathy. Heart. 2000;84 (5): 541-7. <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1729493">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/11040018">Pubmed citation</a><span class="auto"></span>
  • 2. Kremastinos DT, Farmakis D. Iron overload cardiomyopathy in clinical practice. Circulation. 2011;124 (20): 2253-63. <a href="http://dx.doi.org/10.1161/CIRCULATIONAHA.111.050773">doi:10.1161/CIRCULATIONAHA.111.050773</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/22083147">Pubmed citation</a><span class="auto"></span>

Systems changed:

  • Cardiac

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