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CLINICAL STUDY: ENDOCARDIAL FIBROELASTOSIS

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

Manuscript received April 26, 2001; revised manuscript received April 15, 2002, accepted May 15, 2002.

^* Reprint requests and correspondence: Dr. Lisa K. Hornberger, The Hospital for Sick Children, Division of Cardiology, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.
hornberg{at}sickkids.on.ca

OBJECTIVES: This study was designed to document the association of endocardial fibroelastosis (EFE) and maternal autoantibodies.

BACKGROUND: Neonatal lupus erythematosus is associated with the transplacental passage of maternal anti-Ro and anti-La antibodies, leading to complete atrioventricular block (CAVB). In some cases, CAVB is associated with EFE. Isolated EFE may be independently related to maternal anti-Ro and anti-La antibodies.

METHODS: We identified three cases (one fetus and two infants, all female) of isolated EFE in infants born to autoantibody-positive mothers in the absence of CAVB. Demographics, echocardiograms, and pathology were reviewed. Immunohistochemical analyses for immunoglobulin (Ig)G, IgM, IgA, T-cell, B-cell, and terminal deoxynucleoleotidyl transferase-mediated dUTP-biotin nick end-labeling (TUNEL) (test for cell apoptosis) staining were performed on multiple sections of the heart in each case and compared with negative controls.

RESULTS: Two cases died and one received a cardiac transplant. All three cases had histologically confirmed EFE. All cases demonstrated significant diffuse IgG infiltration. To a lesser extent, myocardial tissue was also positive for IgM, CD43, and Granzyme B. None of the specimens were TUNEL positive.

CONCLUSIONS: These are the first reported cases of isolated EFE associated with maternal anti-Ro and anti-La antibodies in the absence of CAVB. The diffuse deposition of IgG and the presence of a T-cell infiltrate throughout the myocardium suggest that the transplacental passage of maternal autoantibodies induces an immune reaction within the myocardium, leading to isolated EFE. Autoantibody-mediated EFE may be an etiologic factor in cases of fetal and neonatal "idiopathic" dilated cardiomyopathy.

Abbreviations and Acronyms   CAVB   complete atrioventricular block   CHF   congestive heart failure   EFE   endocardial fibroelastosis   Ig   immunoglobulin   LV   left ventricular   NLE   neonatal lupus erythematosus   RV   right ventricular   TUNEL   terminal deoxynucleoleotidyl transferase-mediated dUTP-biotin nick end-labeling

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