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Guillain-Barré syndrome, a rare peripheral neuropathy, appears to occur more often in patients who have recently undergone surgery than in the general population. However, the pathophysiologic relationship between surgery and Guillain-Barré syndrome is elusive. Few cases of Guillain-Barré syndrome after cardiac surgery have been reported. Autonomic dysfunction, a serious complication of Guillain-Barré syndrome, has not been previously reported after cardiac surgery. We describe the case of a 71-year-old woman in whom the acute motor axonal neuropathic subtype of Guillain-Barré syndrome developed after mitral valve replacement. Despite plasmapheresis and intravenous immunoglobulin therapy, she died of complications from severe autonomic dysfunction 25 days postoperatively. Recognizing the potential cardiovascular involvement of Guillain-Barré syndrome is important, because patients who undergo cardiac surgery can be vulnerable to autonomic dysfunction in the early postoperative period. [ABSTRACT FROM AUTHOR]