Abstract
OBJECTIVE: To report a rare case of catastrophic antiphospholipid syndrome (CAPS) following surgery for Libman-Sacks endocarditis (LSE), and to clarify the clinical relationship between antiphospholipid syndrome (APS), LSE and CAPS. METHODS: We analysed the clinical course of a patient who developed CAPS after mitral valve replacement surgery for LSE. A literature review was conducted to explore the systemic nature of APS and its cardiac and thrombotic manifestations. RESULTS: A 53-year-old woman initially presented with cerebral infarction and was found to have mitral valve vegetations. Although APS-associated features were present before surgery, a diagnosis was not established at that time. Postoperatively, the patient developed multiple thrombotic events affecting the brain, spleen, kidneys and extremities. Serological testing and clinical deterioration led to the diagnosis of CAPS. Despite supportive therapy, the patient died of multi-organ failure. CONCLUSION: This case highlights the importance of recognising APS as the systemic autoimmune basis of LSE and the need for early immunologic and thrombotic evaluation in patients with unexplained valve lesions. Failure to identify underlying APS before surgery may increase the risk of CAPS and poor outcomes.