Related Experiment Video
Updated: Aug 21, 2026

Sterile Pericarditis in Aachener Minipigs As a Model for Atrial Myopathy and Atrial Fibrillation
Published on: September 24, 2021
Echocardiographic features of atrial myocarditis with giant cells: a case report
Kwan-K Law1, Josepha Binder, Leslie T Cooper
1Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Insights
Wegener's granulomatosis can cause distinct endothelial thickening in the heart. While immunosuppressive therapy resolved this thickening, it did not reverse the associated restrictive cardiomyopathy.
Area of Science:
- Cardiology
- Immunology
- Pathology
Background:
- Restrictive cardiomyopathy presents a diagnostic challenge, often with unclear etiologies.
- Endothelial dysfunction can contribute to cardiac pathology.
Observation:
- Transesophageal echocardiography revealed distinct endothelial thickening of the atrial wall and pulmonary vein orifices in a patient with restrictive cardiomyopathy.
- Echocardiographically guided endomyocardial biopsy showed an inflammatory infiltrate rich in giant cells within the thickened endocardium.
Findings:
- The findings suggested an immune-mediated mechanism for the endocardial thickening.
- Antineutrophil cytoplasmic autoantibody assay was positive, supporting a diagnosis of Wegener's granulomatosis.
- Immunosuppressive therapy led to complete resolution of the endocardial thickening.
Implications:
- Wegener's granulomatosis should be considered in cases of unexplained restrictive cardiomyopathy with specific echocardiographic findings.
- While immunosuppression can resolve inflammatory cardiac manifestations, it may not reverse established myocardial damage.
- This case highlights the importance of integrating echocardiography, biopsy, and serological testing for diagnosing rare cardiac involvements in systemic vasculitis.
Abstract:
We report a case of restrictive cardiomyopathy in which a distinct endothelial thickening of the atrial wall and pulmonary vein orifices was noted on transesophageal echocardiography. Echocardiographically guided endomyocardial biopsy of the thickening revealed an inflammatory infiltrate that was rich in giant cells and provided important clues about an underlying immune mechanism for the pathogenesis. Positive results from the antineutrophil cytoplasmic autoantibody assay supported the diagnosis of Wegener's granulomatosis. After immunosuppressive therapy, the endomyocardial thickening completely resolved, but the restrictive cardiomyopathy did not reverse.
Related Concept Videos
Myocarditis II: Clinical Features and Diagnostic Tests
Myocarditis I: Introduction
Aortic Regurgitation II: Clinical Features and Diagnostic Tests
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Pericarditis II: Clinical Features and Diagnostic Tests
Endocarditis II: Clinical Features of Infective Endocarditis