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If the cause of giant cell myocarditis is unknown, how can it be induced in rats? |
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Can the biopsy results be confused with a different disorder? |
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What is the expected life span of a giant cell myocarditis transplant patient? |
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Why is giant cell myocarditis in a transplant patient not as severe? |
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What type of treatment for giant cell myocarditis is used in a transplant patient? |
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How is giant cell myocarditis detected in a transplant patient? |
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If giant cell myocarditis occurs in a transplant patient how long does it typically take? |
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There are often questions, doubts and guilt experienced by family members. Giant cell myocarditis is a very rare disease that is difficult to diagnose. The symptoms vary from simple fatigue to sudden death. Once a diagnosis is made it is easy to look back and connect these symptoms with giant cell myocarditis. But these same symptoms could have been due to a variety of non-lifethreating conditions. Even if someone were taken to a hospital with minor unexplained symptoms a proper diagnosis would most likely not be made. Myocarditis (enlarged heart) can be detected by a variety of non-invasive techniques. But giant cell myocarditis can only be diagnosed by a heart biopsy. In either case there must be some evidence of heart failure to trigger this type of testing. Typically when a patient exhibits heart failure the disease is attacking the heart very aggressively and the only option is immediate treatment. Even with proper treatment the rate of death or heart transplantation is approximately 70% at one year. The sad truth is that there is
very little if anything that could have been differently.
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The cause of giant cell myocarditis
is not known, but observations in human tissue and experimental
data from a Lewis Rat model suggest that the disease is mediated
by T lymphocytes.[2] |
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If the cause of giant cell myocarditis is unknown, how can it be induced in rats? |
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Experimental giant cell myocarditis
can be produced in the Lewis rat by auto immunization
with myosin.[2]
[3]
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No. Giant cell myocarditis
was first described in 1905 |
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Giant cell myocarditis is a very
rare disease. Many physicians are not familiar with giant cell myocarditis
either. |
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Giant cell myocarditis can only be diagnosed with an endomyocardial (heart) biopsy. |
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Giant Cell Myocarditis Endomyocardial Biopsy
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Can the biopsy results be confused with a different disorder? |
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Giant cell myocarditis can be confused with cardiac sarcoidosis. Therefore all suspected cases should be reviewed by an experienced cardiac pathologist. |
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Cardiac Sarcoidosis Endomyocardial Biopsy
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Giant Cell Myocarditis Endomyocardial Biopsy
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A thin tube called a catheter is inserted into a vein or artery, usually in the groin or neck. The catheter is guided using x-ray or echocardiogram (sonogram for the heart) and placed inside of the heart. Then an instrument called a biotone is inserted into the catheter and used to remove a small section of the heart from the inside. The following picture shows the biotone being used to perform a biopsy. |
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Endomyocardial Biopsy Using a Biotone
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One treatment is to give the patient
a variety of immunosupressive
drugs combined with steroids. Another option is
a heart transplant.
"Giant Cell Myocarditis (GCM) is an uncommon disorder with no proven effective treatment resulting in an 89% cardiac mortality by three years. In an animal model and observational human studies, muromonab-CD3 and cyclosporine effectively treat GCM. Muromonab-CD3 is a monoclonal antibody that causes lymphonpenia by binding the CD3 receptor of human T lymphocytes. Cyclosporine inhibits T lymphocye activation." [4] The goal of immunosupression therapy
is to prolong heart transplantion. The
need for a transplant my be prolonged by months or even
years. Since each case is unique there is not
a typical time. |
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What is the expected life span of a giant cell myocarditis transplant patient? |
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Post-transplantation survival is approximately 71% at
five years despite a 25% rate of giant cell infiltration in the donor heart. To
confirm these findings, a randomized trial of immunosuppression including
muromonab-CD3, cyclosporine, and steroid is underway at the Mayo Clinic in Rochester
Minnesota.. |
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Giant cell myocarditis reoccurs
in approximately 25% of transplant patients. Generally
giant cell myocarditis is not as severe in a transplant
patient. |
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Why is giant cell myocarditis in a transplant patient not as severe? |
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The exact reason is not known.
But the disease may be less aggressive because
the patients are on immunosuppressive drugs to prevent
rejection of the donor heart.. |
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What type of treatment for giant cell myocarditis is used in a transplant patient? |
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The treatment depends to some
degree on the presentation, but may consist of steroids
for 2-3 months. |
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How is giant cell myocarditis detected in a transplant patient? |
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The diagnosis is made by heart
biopsy, which is routinely performed in the post-transplant
patient to look for rejection of the donor heart. |
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If giant cell myocarditis occurs in a transplant patient how long does it typically take to appear? |
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The range of time to recurrence
is wide from 3 weeks to 9 years after transplant with
an average time of about 1 year. |
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