Atrial giant cell myocarditis: a distinctive clinicopathologic entity
Brandon T Larsen1, Joseph J Maleszewski, William D Edwards
1Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
Circulation
|November 28, 2012
Summary
Giant cell myocarditis (GCM) can affect the atria, presenting differently than typical ventricular GCM. This atrial variant shows a more favorable prognosis and distinct clinical features.
Area of Science:
- Cardiology
- Pathology
- Immunology
Background:
- Giant cell myocarditis (GCM) typically presents as fulminant heart failure, requiring aggressive treatment.
- A novel variant of GCM primarily affecting the atria has been identified.
- This atrial GCM variant exhibits distinct clinical features and a more benign clinical course.
Observation:
- Six patients with atrial GCM were identified between 2010-2012.
- Clinical presentations included atrial fibrillation, heart failure, and incidental findings.
- Echocardiography revealed severe atrial dilatation, mitral/tricuspid regurgitation, and atrial wall thickening, with preserved ventricular function.
Findings:
- Histological analysis showed giant cell and lymphocytic infiltrates, cardiomyocyte necrosis, and hypertrophy in atrial tissue.
- Associated findings included interstitial fibrosis, granulomas, eosinophils, and rare neutrophils or vasculitis.
- Patients treated with steroids, cyclosporine, or supportive care showed recovery of exercise tolerance.
Implications:
- Atrial GCM is a distinct clinicopathologic entity with a better prognosis than ventricular GCM.
- Consider atrial GCM in the differential diagnosis of atrial dilatation, especially with atrial wall thickening.
- The efficacy of immunomodulatory therapy for atrial GCM requires further investigation.
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