Atrial giant cell myocarditis

A distinctive clinicopathologic entity

Brandon Larsen, Joseph Maleszewski, William D. Edwards, Leslie T Jr. Cooper, Richard E. Sobonya, V. Eric Thompson, Simon G. Duckett, Charles R. Peebles, Iain A. Simpson, Henry D. Tazelaar

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

Background-Giant cell myocarditis (GCM) typically causes fulminant heart failure, arrhythmias, or heart block, necessitating aggressive immunosuppression, ventricular assist device insertion, or cardiac transplantation. We describe a novel variant of GCM, primarily involving the atria, that displays distinctive clinical features and follows a more benign course than ventricular GCM. Methods and Results-We identified 6 patients (median age 67.5 years, 4 male) with atrial GCM in our pathology consultation practices from 2010 to 2012. Clinical history, imaging, and pathology materials were reviewed. Clinically, 4 patients had atrial fibrillation, 1 had acute heart failure, and 1 had incidental disease at autopsy. Among the 5 living patients, echocardiography revealed severe atrial dilatation (5 cases), mitral/tricuspid regurgitation (5), atrial mural thrombus (3), atrial wall thickening (2), and atrial hypokinesis (2). Ventricular function was preserved in all 5. Histological review of surgically resected atria showed giant cell and lymphocytic infiltrates, lymphocytic myocarditis-like foci, cardiomyocyte necrosis, and cardiomyocyte hypertrophy in all cases. Other features included interstitial fibrosis (5), poorly-formed granulomas (4), eosinophils (4), neutrophils (1), and vasculitis (1). Treatment consisted of steroids and cyclosporine (1), pacemaker placement for sick sinus syndrome (1), and supportive care (3). All 5 living patients returned to baseline exercise tolerance after 6 to 16 weeks of follow-up. Conclusions-Atrial GCM represents a distinct clinicopathologic entity with a more favorable prognosis than classic ventricular GCM. This disorder should be included in the differential diagnosis of atrial dilatation, particularly when associated with atrial wall thickening. The utility of immunomodulatory therapy for this condition remains unknown.

Original languageEnglish (US)
Pages (from-to)39-47
Number of pages9
JournalCirculation
Volume127
Issue number1
DOIs
StatePublished - Jan 1 2013

Fingerprint

Myocarditis
Giant Cells
Cardiac Myocytes
Dilatation
Heart Failure
Pathology
Sick Sinus Syndrome
Tricuspid Valve Insufficiency
Heart-Assist Devices
Heart Block
Exercise Tolerance
Ventricular Function
Immunomodulation
Mitral Valve Insufficiency
Heart Transplantation
Vasculitis
Granuloma
Eosinophils
Immunosuppression
Atrial Fibrillation

Keywords

  • heart enlargement
  • myocarditis
  • sarcoidosis

ASJC Scopus subject areas

  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine

Cite this

Atrial giant cell myocarditis : A distinctive clinicopathologic entity. / Larsen, Brandon; Maleszewski, Joseph; Edwards, William D.; Cooper, Leslie T Jr.; Sobonya, Richard E.; Eric Thompson, V.; Duckett, Simon G.; Peebles, Charles R.; Simpson, Iain A.; Tazelaar, Henry D.

In: Circulation, Vol. 127, No. 1, 01.01.2013, p. 39-47.

Research output: Contribution to journalArticle

Larsen, B, Maleszewski, J, Edwards, WD, Cooper, LTJ, Sobonya, RE, Eric Thompson, V, Duckett, SG, Peebles, CR, Simpson, IA & Tazelaar, HD 2013, 'Atrial giant cell myocarditis: A distinctive clinicopathologic entity', Circulation, vol. 127, no. 1, pp. 39-47. https://doi.org/10.1161/CIRCULATIONAHA.112.128900
Larsen, Brandon ; Maleszewski, Joseph ; Edwards, William D. ; Cooper, Leslie T Jr. ; Sobonya, Richard E. ; Eric Thompson, V. ; Duckett, Simon G. ; Peebles, Charles R. ; Simpson, Iain A. ; Tazelaar, Henry D. / Atrial giant cell myocarditis : A distinctive clinicopathologic entity. In: Circulation. 2013 ; Vol. 127, No. 1. pp. 39-47.
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