An Autopsy Study of Sudden Cardiac Death Associated with Lyme Carditis
Atis Muehlenbachs∗,∗,'Correspondence information about the author Atis MuehlenbachsEmail the author 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.
Supported by the Centers for Disease Control and Prevention.
The findings and conclusions herein are those of the authors and do not necessarily represent the official position of the Centers for Disease Control and Prevention.
Disclosures: T.J.S. and G.S.R. are employed by, and have stock options in CryoLife, Inc.
Current address of J.D.F., Division of General Surgery, Stanford University, Palo Alto, CA; of D.L.N., Departments of Pathology and Laboratory Medicine, University of California at San Francisco, San Francisco, CA; of J.A.P., Homer Stryker MD School of Medicine, Western Michigan University, Kalamazoo, MI.
Published by Elsevier Inc. on behalf of American Society for Investigative Pathology.
Atis Muehlenbachs∗,∗,'Correspondence information about the author Atis MuehlenbachsEmail the author 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.
Supported by the Centers for Disease Control and Prevention.
The findings and conclusions herein are those of the authors and do not necessarily represent the official position of the Centers for Disease Control and Prevention.
Disclosures: T.J.S. and G.S.R. are employed by, and have stock options in CryoLife, Inc.
Current address of J.D.F., Division of General Surgery, Stanford University, Palo Alto, CA; of D.L.N., Departments of Pathology and Laboratory Medicine, University of California at San Francisco, San Francisco, CA; of J.A.P., Homer Stryker MD School of Medicine, Western Michigan University, Kalamazoo, MI.
Published by Elsevier Inc. on behalf of American Society for Investigative Pathology.
