Cardiac Path Online

Pathology of Cardiac Hemochromatosis

Dr Sampurna Roy MD

 

                                                                                                                      

 

The heart is almost always affected in idiopathic (primary) hemochromatosis.

In addition cardiac iron deposits occur in secondary hemochromatosis, which occurs, for example, in patients who have received multiple blood transfusions for treatment of anemia.

The degree of involvement may be slight and without evidence of gross changes, but often it is sufficient to produce a brownish discoloration of the myocardium.

The deposits are more prominent in the subendothelial than in the subepicardial zone.

Microscopically, hemosiderin granules are identified within myocardial fibers and occasionally in the connective tissue cells.

In the myocytes, the iron is deposited in sarcoplasmic siderin granules, which are distinct from lipofuscin granules.

Varying degrees of degeneration, edema, and fibrosis of the myocardium can be seen.

Electrocardiographic changes and even cardiac failure may occur.

Arrhythmias may occur as a result of iron deposits in the fibers of the conduction system.

Further reading:

Endomyocardial biopsy in hemochromatosis

Reversal of severe biventricular dysfunction from cardiac hemochromatosis with iron removal.

Unsuspected hereditary hemochromatosis at forensic autopsy: its presentation, confirmation, and implications.

On myocardial siderosis and left ventricular dysfunction in hemochromatosis.

Hemochromatosis (HFE) gene mutations and risk of gastric cancer in the European Prospective Investigation into  Cancer and Nutrition (EPIC) study.

Reversal of severe biventricular dysfunction from cardiac hemochromatosis with iron removal.

Iron overload decreases CaV1.3-dependent L-type Ca2+ currents leading to bradycardia, altered electrical conduction, and atrial fibrillation.

Cardiac involvement in hemochromatosis.

Cardiac iron deposition in idiopathic hemochromatosis

Fatal "iron heart" in an adolescent

Unsuspected hereditary hemochromatosis at forensic autopsy: its presentation, confirmation, and implications.

Mechanical ventricular assistance in heart failure: pathology of the cardiac apex removed during device implantation.

HFE mutations in idiopathic dilated cardiomyopathy.

Early diagnosis of hemochromatosis-related cardiomyopathy with magnetic resonance imaging.

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

 


 

 

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