Contents
Definition
Giant cell myocarditis is a rare type of severe heart muscle inflammation of unknown cause which is occasionally related to rheumatic disease. The disease often causes rapidly increasing heart failure in younger or middle-aged people. Giant cell myocarditis is difficult to recognise, so a tissue sample from the heart muscle is necessary for a secure diagnosis. A combination of various immunosuppressive drugs and early diagnosis reduces the need for heart transplantation and the risk of early death (reference: Bang V, 2020). In approximately one in five cases, there is a known rheumatic or other condition at the same time Autoimmune disease.
Occurrence
The disease is rare (reference: Vaideesvar P, 2013). Men and women are attacked equally often. Most are aged 40-60, approx. 6% are under 19 years of age. Concomitant autoimmune disease (connective tissue disorders and others) occur in approximately 20% of cases (references: Blauwet LA, 2012; Bang V, 2020).
Symptoms
Giant cell myocarditis has no completely typical characteristics at the beginning. Symptoms that may give rise to suspicion of the condition are incipient heart failure without prompting in a relatively young person. Heart rhythm disorder with an irregular pulse is the second most common symptom, and some make their debut with one heart attack-similar disease picture (reference: Cooper LT, 1997). It is typical that the symptoms increase already within 1-2 weeks despite usual treatment (reference: Bang V, 2020).
Examinations
Medical history are often otherwise unexplained symptoms of new heart disease (see above) in a relatively young person.
Clinical examination may reveal signs of heart failure and/or an irregular or excessively slow pulse.
Blood tests includes CRP, lowering reaction (SR), cell counts, liver-kidney, metabolism/thyroid function tests, CK and troponin from muscle/myocardium and urine prick, but also ANA and other markers if concurrent inflammatory/autoimmune disease is suspected. NT-proBNP will indicate whether heart failure is present.
Special heart examinations such as EKG, echocardiography and cardiac catheter examination often give rise to suspicion of the disease.
Tissue sample from the heart muscle can be taken using a heart catheter that is pushed in via blood vessels, so that open surgical intervention is not necessary. Microscopic examination of heart muscle tissue will show inflammatory changes with so-called multinuclear giant cells, without signs of infection.
Treatment
Immunosuppressive treatment can have effect and better survival. Combination of ciclosporin (Sandimmun) og prednisolone is used. Heart Transplant must still be assessed. In an acute phase of illness, mechanical assistance for blood circulation in a well-equipped specialist department may be necessary. Many need an implanted cardioverter-defibrillator (ICD) that interrupts serious heart rhythm disturbances. Nevertheless, a heart transplant is often necessary within a year (reference: Kandoline R, 2012).
