Infective endocarditis of the right atrioventricular valve and transvenous pacing lead in a patient with Down syndrome

An 11-year-old with Down syndrome, autism, prior atrioventricular septal defect repair, and a transvenous pacemaker presented with prolonged fever and raised inflammatory markers. Eight years prior, she had a group A streptococcal sepsis with infection of an epicardial pacemaker. Echocardiography demonstrated a large vegetation on the right atrioventricular valve, with encasement of the pacemaker lead. Following treatment with intravenous antibiotics, she underwent a right atrioventricular valve repair, vegetation excision, and epicardial pacemaker placement. Post-operative recovering was uneventful. This case highlights the complex, multidisciplinary management of device-related endocarditis in Down syndrome and in CHD.