Isolated tricuspid valve Staphylococcus lugdunensis endocarditis in patient with a KBG syndrome

Both KBG syndrome (approximately 50 patients worldwide) and isolated tricuspid valve Staphylococcus lugdunensis endocarditis are very rare entities. The KBG syndrome is a multiple congenital anomaly characterized by short stature, distinctive craniofacial features, and neurologic/developmental/cognitive delay and is only associated to congenital heart defects in 9% of patients. Staphylococcus lugdunesis is an aggressive cause of infective endocarditis. Herein is described a case of a patient presenting both diseases, despite the absence of any known infection, congenital heart defect, heart device or any other entry port which could explain this scenario. The unusual findings in this young patient raised questions regarding the, as-yet unexplained, etiopathogenesis of the KBG syndrome, the possibility of it being related to this rare and concerning clinical presentation and the unclear and undefined management and surgical approach associated to right side endocarditis.