Giant cell myocarditis: from immune pathogenesis to contemporary management.
Endomyocardial biopsy detects giant cell myocarditis with 85% sensitivity
Giant cell myocarditis: from immune pathogenesis to contemporary management.
Giant cell myocarditis (giant cell myocarditis) is a rare but devastating inflammatory cardiac disease characterized by rapid hemodynamic deterioration, malignant arrhythmias, and high rates of death or heart transplantation.
Without immunosuppressive treatment, median transplant-free survival is approximately three months.
Viral infections and environmental factors have been implicated as potential triggers.
Giant cell myocarditis typically affects middle-aged adults, with concomitant non-cardiac autoimmune diseases present in approximately 20% of cases.
Management requires prompt initiation of combination immunosuppressive therapy-typically corticosteroids combined with T cell-targeted agents such as cyclosporine and azathioprine or tacrolimus and mycophenolate mofetil, which has substantially improved survival in contemporary registries.
This review provides a comprehensive overview of the pathogenesis, diagnosis, and current management of giant cell myocarditis and highlights priorities for future research, including noninvasive diagnostic tools and standardized treatment protocols.