Cardiac Tropism of Borrelia burgdorferi An Autopsy Study of Sudden Cardiac Death Associated with Lyme Carditis

Atis Muehlenbachs, Brigid C. Bollweg, Thadeus J. Schulz, Joseph D. Forrester, Marlene DeLeon Carnes, Claudia Molins, Gregory S. Ray, Peter M. Cummings, Jana M. Ritter, Dianna M. Blau, Thomas A. Andrew, Margaret Prial, Dianna L. Ng, Joseph A. Prahlow, Jeanine H. Sanders, Wun Ju Shieh, Christopher D. Paddock, Martin E. Schriefer, Paul Mead, Sherif R. Zaki

Fatal Lyme carditis caused by the spirochete Borrelia burgdorferi rarely is identified. Here, we describe the pathologic, immunohistochemical, and molecular findings of five case patients. These sudden cardiac deaths associated with Lyme carditis occurred from late summer to fall, ages ranged from young adult to late 40s, and four patients were men. Autopsy tissue samples were evaluated by light microscopy, Warthin-Starry stain, immunohistochemistry, and PCR for B. burgdorferi, and immunohistochemistry for complement components C4d and C9, CD3, CD79a, and decorin. Post-mortem blood was tested by serology. Interstitial lymphocytic pancarditis in a relatively characteristic road map distribution was present in all cases. Cardiomyocyte necrosis was minimal, T cells outnumbered B cells, plasma cells were prominent, and mild fibrosis was present. Spirochetes in the cardiac interstitium associated with collagen fibers and co-localized with decorin. Rare spirochetes were seen in the leptomeninges of two cases by immunohistochemistry. Spirochetes were not seen in other organs examined, and joint tissue was not available for evaluation. Although rare, sudden cardiac death caused by Lyme disease might be an under-recognized entity and is characterized by pancarditis and marked tropism of spirochetes for cardiac tissues.