What is hemochromatosis (iron overload cardiomyopathy)?

Updated: Nov 28, 2018
  • Author: Vinh Q Nguyen, MD; Chief Editor: Gyanendra K Sharma, MD, FACC, FASE  more...
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Answer

Iron deposition in the myocardium initially manifests as diastolic dysfunction from a restrictive pathophysiology that progresses to systolic dysfunction. [63]  Iron accumulates first in the ventricular myocardium and then the atrial myocardium. [64] As iron itself is proarrhythmic, its involvement in the conduction system may explain the propensity for hemochromatosis toward atrial or ventricular tachyarrhythmias. [65]  Iron deposition in the conduction system may cause bradyarrhythmias, warranting placement of pacemakers. [64]

Iron overload is characterized by a transferrin saturation above 55% and a transferrin level over 200 ng/mL for women and over 300 ng/mL for men (on the basis of the 2005 American College of Physicians [ACP] guidelines). [66, 67] However, the level of ferritin in which myocardial deposition is detected is not defined. [63]

As noted earlier, EMB has low sensitivity for hemochromatosis due to the patchy involvement of the myocardium. [12] Echocardiographic findings include ventricular dilatation and restrictively cardiomyopathy. [63] Iron removal may reverse these findings.


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