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.

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