A severe case of Austrian syndrome

Abstract

A 43-year-old woman was referred to our echocardiography laboratory with recent onset, progressive exertional dyspnea. She had been hospitalized 2 months earlier while pregnant for pneumococcal pneumonia with meningitis and severe aortic regurgitation caused by aortic valve endocarditis, a clinical triad first described in 1956 and referred to as Austrian syndrome. In that setting, after caesarean delivery, aortic valve replacement (AVR) was performed. Mother and child recovered well. At the current presentation, 2 months after AVR and after 51 days of antibiotic therapy according to susceptibility testing, transesophageal echocardiography revealed an aortic annular abscess with perforation into the left atrium (Figure 1); the hemodynamic situation resembled severe mitral regurgitation (Figure 2). The AVR and the anterior mitral leaflet were excised, and AVR and mitral valve replacement were performed. No pathogens could be detected in blood cultures, tissue biopsy, or intraoperative smear. The patient recovered well. Unusual infections caused by Streptococcus pneumoniae were frequent in the preantibiotic age but rapidly declined after the introduction of penicillin. The incidence of pneumococcal endocarditis now accounts for less than 3% of all cases and most often occur in patients with risk factors, especially alcoholism, whereas underlying valvular heart disease does not seem to be a strong risk factor. The aortic valve is most commonly involved with a high rate of local and systemic complications and substantial mortality. High suggestion for cardiac involvement should be raised in patients with systemic pneumococcal disease.