Endocardial fibroelastosis associated with maternal anti-Ro and anti-La antibodies in the absence of atrioventricular block
Lynne E. Nield, MD*,
Earl D. Silverman, MD ,
Jeffrey F. Smallhorn, MD*,
Glenn P. Taylor, MD ,
J. Brendan,
M. Mullen, MD ,
Leland N. Benson, MD* and
Lisa K. Hornberger, MD*,*
* Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children and the Research Institute, Toronto, Canada
Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children and the Research Institute, Toronto, Canada
Division of Pathology, Department of Pediatrics, The Hospital for Sick Children and the Research Institute, Toronto, Canada
Department of Pathology and Laboratory Medicine, Mount Sinai Hospital, the University of Toronto Faculty of Medicine, Toronto, Canada
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Figure 1 Transthoracic echocardiogram at the time of hospital admission for congestive heart failure demonstrating a dilated left ventricle (LV) with areas of endocardial fibroelastosis along the endocardial surface of the LV, mitral valve, and papillary muscles. LA = left atrium.
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Figure 2 Pathologic specimen of the explanted heart illustrating diffuse, severe endocardial fibroelastosis in the left ventricle, mitral valve and papillary muscles, and the globular-shaped heart.
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Figure 3 Paraffin sections of case 1 using alkaline phosphatase-conjugated antibodies directed against (staining methods as outlined in Methods section): (A) human immunoglobulin (Ig)G demonstrating diffuse deposition of IgG; (B) human IgM demonstrating focal areas of deposition; (C) negative terminal deoxynucleoleotidyl transferase-mediated dUTP-biotin nick end-labeling staining for apoptosis; (D) negative staining for pan B cell marker (CD 20); (E) focal areas of pan T cell marker (CD 43); and (F) focal area of Granzyme B positive staining (magnification 25 x 10 x 1.25).
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Figure 4 Paraffin sections of case 1 and normal control case using alkaline phosphatase conjugated antibodies directed against (staining methods as outlined in Methods section): (A) human immunoglobulin (Ig)G demonstrating dense, diffuse deposition of IgG with loss of nuclei and myocardial disarray; (B) human IgM demonstrating focal areas of deposition; (C) human IgG in control case demonstrating mild, diffuse IgG deposition with a normal myocardial muscle pattern with normal, multiple nuclei; (D) human IgM in control case showing normal myocardial muscle pattern with normal, multiple without deposition of IgM (magnification 20 x 0.5).
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