A49-42 Austrian Syndrome in an Immunocompetent Host

Abstract Introduction Austrian Syndrome describes a rare triad of pneumonia, endocarditis, and meningitis. It is typically caused by Streptococcus pneumoniae and usually occurs in immunocompetent or asplenic patients. Description A 68-year-old male with a history of a bicuspid aortic valve, a bioprosthetic aortic valve replacement, and a remote alcohol use disorder presented to the emergency department with fatigue, myalgias, and dyspnea and diagnosed with community acquired pneumonia. Initial labs were notable for a white blood cell count of 24. Chest x-ray revealed right-sided apical opacities. A transthoracic echocardiogram demonstrated mildly decreased left ventricular systolic function with a normally functioning bioprosthetic valve. He was admitted to the cardiology service for a transesophageal echocardiogram (TEE) to rule out endocarditis. The next day, his neurologic exam acutely worsened, with inability to follow directions, and he then developed expressive aphasia. A CT head showed no abnormalities. Lumbar puncture revealed grossly purulent cerebrospinal fluid (CSF) with a neutrophilic predominant cell count of 8,897, protein of 647, and glucose less than 5. Empiric coverage with ceftriaxone and vancomycin for bacterial meningitis was started. CSF and blood cultures grew Streptococcus pneumoniae. His TEE showed prosthetic valve endocarditis and a paravalvular abscess. His course was further complicated by a cerebrovascular accident, with brain imaging showing multiple acute infarcts. He underwent a Commando Procedure with double replacement of the aortic and mitral valves and reconstruction of the aortomitral fibrous body. His mental status never improved to his baseline, and he was discharged to a long-term acute care hospital. He died one month following hospital discharge. Discussion Austrian syndrome is a triad of pneumonia, endocarditis, and meningitis, typically occurring in immunocompromised or asplenic patients, and caused by invasive pneumococcal disease. Additional risk factors include advanced age, alcoholism, and intravenous drug use. Since the introduction of beta-lactam antibiotics in the 1940s, it is very rarely seen but can have mortality rates as high as 28% despite appropriate treatment. There is no documented association between pneumococcal endocarditis and valve replacement, though bioprosthetic valves incur a higher risk of infectious endocarditis than mechanical heart valves. Our patient had an intact spleen, no indication of an immunocompromised status, and had received the Prevnar 20 vaccine three years prior to admission. He also quit drinking alcohol several years prior and had no history of intravenous drug use. His rapid decline highlights the need for early detection and treatment of invasive pneumococcal disease, especially Austrian Syndrome. This abstract is funded by: None