Idiopathic giant-cell myocarditis (IGCM) is a cardiovascular disease of the muscle of the heart (
myocardium).
The condition is rare;
however, it is often fatal and there is no proven cure because of the unknown nature of the disorder.
IGCM frequently leads to death with a high rate of about 70% in first year. A patient with IGCM typically presents with symptoms of
heart failure, although some may present initially with
ventricular arrhythmia
Arrhythmias, also known as cardiac arrhythmias, heart arrhythmias, or dysrhythmias, are irregularities in the heartbeat, including when it is too fast or too slow. A resting heart rate that is too fast – above 100 beats per minute in adults ...
or
heart block
Heart block (HB) is a disorder in the heart's rhythm due to a fault in the natural pacemaker. This is caused by an obstruction – a block – in the electrical conduction system of the heart. Sometimes a disorder can be inherited. Despite the se ...
. Median age from the time the disease is diagnosed to the time of death is approximately 6 months. 90% of patients are either deceased by the end of 1 year or have received a heart transplant. Diagnosis is made by
endomyocardial biopsy during
heart catheterization
Cardiac catheterization (heart cath) is the insertion of a catheter into a chamber or vessel of the heart. This is done both for diagnostic and interventional purposes.
A common example of cardiac catheterization is coronary catheterization tha ...
. Biopsy shows
multinucleated giant cells and thus the name. While previously cases universally required heart transplantation, recent studies show that two-thirds of patients can survive past one year with high doses of immunosuppressants such as prednisone and cyclosporine. The transplanted heart has a high chance of disease recurrence. Compared to lymphocytic (presumed
viral) myocarditis, giant cell myocarditis is much more severe with much more rapid progression.
It is suggested to be caused by
T-lymphocytes
A T cell is a type of lymphocyte. T cells are one of the important white blood cells of the immune system and play a central role in the adaptive immune response. T cells can be distinguished from other lymphocytes by the presence of a T-cell r ...
.
See also
*
Idiopathic
An idiopathic disease is any disease with an unknown cause or mechanism of apparent spontaneous origin. From Greek ἴδιος ''idios'' "one's own" and πάθος ''pathos'' "suffering", ''idiopathy'' means approximately "a disease of its own kin ...
*
Giant cell
A giant cell (also known as multinucleated giant cell, or multinucleate giant cell) is a mass formed by the union of several distinct cells (usually histiocytes), often forming a granuloma. Although there is typically a focus on the pathologica ...
*
Myocarditis
References
External links
Heart diseases
Idiopathic diseases
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