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Idiopathic giant-cell myocarditis--natural history and treatment. Multicenter Giant Cell Myocarditis Study Group
L T Cooper1, G J Berry, R Shabetai
1Department of Medicine, University of California at San Diego Medical Center, USA. cooper.leslie@mayo.edu
Insights
Giant-cell myocarditis is a rare, fatal heart condition. Immunosuppressive therapy significantly improves survival, and heart transplantation is the preferred treatment despite recurrence risks.
Area of Science:
- Cardiology
- Immunology
- Pathology
Background:
- Idiopathic giant-cell myocarditis is a rare, often fatal cardiac disorder.
- Understanding its natural history and treatment effects is crucial.
Purpose of the Study:
- To define the natural history of idiopathic giant-cell myocarditis.
- To evaluate the impact of various treatments on patient outcomes.
Main Methods:
- A multicenter database was used to identify 63 patients with idiopathic giant-cell myocarditis.
- Data on demographics, presenting symptoms, associated conditions, and treatment outcomes were collected and analyzed.
Main Results:
- The condition predominantly affects relatively young, healthy adults, with common presentations including heart failure and ventricular arrhythmias.
- Survival was significantly worse compared to lymphocytic myocarditis, with an 89% rate of death or cardiac transplantation and a median survival of 5.5 months.
- Patients treated with immunosuppressive therapy (corticosteroids and/or cyclosporine, azathioprine) survived an average of 12.3 months, compared to 3.0 months for those untreated (P=0.001).
- Heart transplantation offered improved survival, but 26% of transplanted hearts showed recurrence, and 1 patient died from it.
Conclusions:
- Giant-cell myocarditis is a severe disease, typically leading to death from heart failure or arrhythmias without intervention.
- While heart transplantation is the treatment of choice, the risk of fatal recurrence necessitates careful consideration.
- Immunosuppressive therapy demonstrates a significant survival benefit.
Background:
Idiopathic giant-cell myocarditis is a rare and frequently fatal disorder. We used a multicenter data base to define the natural history of giant-cell myocarditis and the effect of treatment.
Methods:
We identified 63 patients with idiopathic giant-cell myocarditis through journal announcements and direct mailings to cardiovascular centers worldwide.
Results:
The patients consisted of 33 men and 30 women with an average age of 42.6 years; 88 percent were white, 5 percent were black, 5 percent were Southeast Asian or Indian, and 2 percent were Middle Eastern. Most presented with congestive heart failure (47 patients, or 75 percent), ventricular arrhythmia (9 patients, or 14 percent), or heart block (3 patients, or 5 percent), although in some cases the initial symptoms resembled those of acute myocardial infarction (4 patients). Nineteen percent had associated autoimmune disorders. The rate of survival was worse than among 111 patients with lymphocytic myocarditis in the Myocarditis Treatment Trial (P<0.001); among our patients, the rate of death or cardiac transplantation was 89 percent, and median survival was only 5.5 months from the onset of symptoms. The 22 patients treated with corticosteroids and cyclosporine, azathioprine, or both therapies survived for an average of 12.3 months, as compared with an average of 3.0 months for the 30 patients who received no immunosuppressive therapy (P=0.001). Of the 34 patients who underwent heart transplantation, 9 (26 percent) had a giant-cell infiltrate in the transplanted heart and 1 died of recurrent giant-cell myocarditis.
Conclusions:
Giant-cell myocarditis is a disease of relatively young, predominantly healthy adults. Patients usually die of heart failure and ventricular arrhythmia unless cardiac transplantation is performed. Despite the possibility of fatal disease recurrence, transplantation is the treatment of choice for most patients.