Cardiac involvement in hemochromatosis typically occurs with primary hemochromatosis, as the organ is usually spared in the secondary form of the disease.
For a general discussion, and for links to other system specific manifestations, please refer to the article on hemochromatosis.
Cardiac involvement occurs in approximately 15-20% of the patients with hemochromatosis.
Manifestations depend on the extent of iron deposition and include:
- pedal edema
- features of congestive heart failure
Hemochromatosis can result in an iron overload cardiomyopathy.
Findings are non-specific 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 hemosiderin 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.
- 1.Cheong B, Huber S, Muthupillai R et-al. Evaluation of myocardial iron overload by T2* cardiovascular magnetic resonance imaging. Tex Heart Inst J. 2006;32 (3): 448-9. Free text at pubmed - Pubmed citation
- 2. McCarthy GM, Crowe J, McCarthy CJ et-al. Hereditary hemochromatosis: a common, often unrecognized, genetic disease. Cleve Clin J Med. 2002;69 (3): 224-6, 229-30, 232-3 passim. Cleve Clin J Med (abstract) - doi:10.3949/ccjm.69.3.224 - Pubmed citation
- 3. Passen EL, Rodriguez ER, Neumann A et-al. Images in cardiovascular medicine. Cardiac hemochromatosis. Circulation. 1996;94 (9): 2302-3. Circulation (full text) - doi:10.1161/01.CIR.94.9.2302 - Pubmed citation
- 4. Ptaszek LM, Price ET, Hu MY et-al. Early diagnosis of hemochromatosis-related cardiomyopathy with magnetic resonance imaging. J Cardiovasc Magn Reson. 2006;7 (4): 689-92. Pubmed citation
- 5. Blankenberg F, Eisenberg S, Scheinman MN et-al. Use of cine gradient echo (GRE) MR in the imaging of cardiac hemochromatosis. J Comput Assist Tomogr. 1994;18 (1): 136-8. Pubmed citation