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Libman–Sacks endocarditis is rare in children and adolescents, more so as a first manifestation of systemic lupus erythematosus. Currently, sterile verrucous lesions of Libman–Sacks endocarditis are recognised as a cardiac manifestation of both systemic lupus erythematosus and antiphospholipid syndrome. They are clinically silent in a majority of the cases. The presence of antiphospholipid antibodies in systemic lupus erythematosus is associated with three times higher prevalence of mitral valve nodules and significant mitral regurgitation. We present the case of isolated mitral regurgitation with abnormal looking mitral valve, detected in early childhood, which deteriorated to a severe degree in the next decade and was diagnosed as Libman–Sacks endocarditis after surgical repair from histopathology. The full-blown clinical spectrum of systemic lupus erythematosus with antiphospholipid antibodies was observed several weeks after cardiac surgery. We discuss the atypical course of Libman–Sacks endocarditis with follow-up for 10 years, along with a review of the literature.
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